Contexts stringlengths 0 32.3k | Questions stringlengths 14 191 | Answers stringlengths 0 29.1k |
|---|---|---|
The rectum is part of
the body’s digestive system. The
digestive system takes in nutrients (vitamins, minerals,
carbohydrates, fats, proteins, and water) from foods and helps pass waste
material out of the body. The digestive system is made up of the
esophagus,
stomach, and the
small and
large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6 to 8 inches long. The anal canal ends at the anus (the opening of the large intestine to the
outside of the body).EnlargeAnatomy of the lower gastrointestinal (digestive) system showing the colon, rectum, and anus. Other organs that make up the digestive system are also shown. For more information about rectal cancer, see: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk to your doctor if you think you may be at risk for colorectal cancer. Risk factors for colorectal cancer include the following: Older age is a main risk factor for most cancers. The chance of getting cancer increases as you get older. These and other signs and symptoms may be caused by rectal cancer or by other conditions. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the
rectum or to other parts of the body
is called staging. The information gathered from the
staging process determines the stage of the disease. It is important
to know the stage in
order to plan treatment. The following tests and
procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if rectal cancer spreads to the lung, the cancer cells in the lung are actually rectal cancer cells. The disease is metastatic rectal cancer, not lung cancer. In stage 0 rectal cancer, abnormal cells are found in the mucosa (innermost layer) of the rectum wall. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in
situ. In stage I rectal cancer, cancer has formed in the mucosa (innermost layer) of the rectum wall and has spread to the submucosa (layer of tissue next to the mucosa) or to the muscle layer of the rectum wall. Stage II rectal cancer is divided into stages IIA, IIB, and IIC. Stage III rectal cancer is divided into stages IIIA, IIIB, and IIIC. In stage IIIA, cancer has spread: In stage IIIB, cancer has spread: In stage IIIC, cancer has spread: Stage IV rectal cancer is divided into stages IVA, IVB, and IVC. The cancer may come back in the
rectum or in other parts of the
body, such as the colon,
pelvis, liver, or lungs. Different types of treatment are available for patients with
rectal cancer. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment.
Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Surgery is the most
common treatment for all stages of rectal cancer. The
cancer is removed using one of the following types of surgery: After the cancer is removed, the surgeon will either: Radiation therapy and/or chemotherapy may be given before surgery to shrink the tumor, make it easier to remove the cancer, and help with bowel control after surgery. Treatment given before surgery is called neoadjuvant therapy. After all the cancer that can be seen at the
time of the surgery is removed, some patients may be given radiation therapy and/or chemotherapy after surgery to kill any cancer
cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant
therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Short-course preoperative radiation therapy is used in some types of rectal cancer. This treatment uses fewer and lower doses of radiation than standard treatment, followed by surgery several days after the last dose. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat cancer that has spread to the liver. This is done by blocking the hepatic artery (the main artery that supplies blood to the liver) and injecting anticancer drugs between the blockage and the liver. The liver’s arteries then carry the drugs into the liver. Only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on what is used to block the artery. The liver continues to receive some blood from the hepatic portal vein, which carries blood from the stomach and intestine. The way the chemotherapy is given depends on the type and stage of the cancer being treated. For more information, see Drugs Approved for Colon and Rectal Cancer. Active surveillance is closely following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests to check if the cancer is growing. When the cancer begins to grow, treatment is given to cure the cancer. Tests include the following: Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Types of targeted therapies used in the treatment of rectal cancer include the following: There are different types of monoclonal antibody therapy: For more information, see Drugs Approved for Colon and Rectal Cancer. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. Immune checkpoint inhibitor therapy: Immune checkpoint inhibitors block proteins called checkpoints that are made by some types of immune system cells, such as T cells, and some cancer cells. These checkpoints help keep immune responses from being too strong and sometimes can keep T cells from killing cancer cells. When these checkpoints are blocked, T cells can kill cancer cells better. They are used to treat some patients with metastatic colorectal cancer. There are two types of immune checkpoint inhibitor therapy: For more information, see Drugs Approved for Colon and Rectal Cancer. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. After treatment for rectal cancer, a blood test to measure amounts of
carcinoembryonic antigen (a
substance in the blood that may be increased when cancer is present) may be
done to see if the cancer has come back. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of stage 0 may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of stage I rectal
cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of stage II and stage III rectal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of stage IV and recurrent rectal cancer may include the following: Treatment of rectal cancer that has spread to other organs depends on where the cancer has spread. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about rectal cancer, see: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of rectal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Rectal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389378] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Rectal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Rectal Cancer ? | Other types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Urethral Cancer ? | Key Points
- Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra. - There are different types of urethral cancer that begin in cells that line the urethra. - A history of bladder cancer can affect the risk of urethral cancer. - Signs of urethral cancer include bleeding or trouble with urination. - Tests that examine the urethra and bladder are used to detect (find) and diagnose urethral cancer. - Certain factors affect prognosis (chance of recovery) and treatment options.
Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra.
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1 inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women.
There are different types of urethral cancer that begin in cells that line the urethra.
These cancers are named for the types of cells that become malignant (cancer): - Squamous cell carcinoma is the most common type of urethral cancer. It forms in cells in the part of the urethra near the bladder in women, and in the lining of the urethra in the penis in men. - Transitional cell carcinoma forms in the area near the urethral opening in women, and in the part of the urethra that goes through the prostate gland in men. - Adenocarcinoma forms in the glands that are around the urethra in both men and women. Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed.
A history of bladder cancer can affect the risk of urethral cancer.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: - Having a history of bladder cancer. - Having conditions that cause chronic inflammation in the urethra, including: - Sexually transmitted diseases (STDs), including human papillomavirus (HPV), especially HPV type 16. - Frequent urinary tract infections (UTIs). |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Urethral Cancer ? | Signs of urethral cancer include bleeding or trouble with urination. These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: - Trouble starting the flow of urine. - Weak or interrupted ("stop-and-go") flow of urine. - Frequent urination, especially at night. - Incontinence. - Discharge from the urethra. - Bleeding from the urethra or blood in the urine. - A lump or thickness in the perineum or penis. - A painless lump or swelling in the groin. |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Urethral Cancer ? | Tests that examine the urethra and bladder are used to detect (find) and diagnose urethral cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Digital rectal exam : An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual. - Urine cytology : A laboratory test in which a sample of urine is checked under a microscope for abnormal cells. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and white blood cells. If white blood cells (a sign of infection) are found, a urine culture is usually done to find out what type of infection it is. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ureteroscopy : A procedure to look inside the ureter and renal pelvis to check for abnormal areas. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis. A tool may be inserted through the ureteroscope to take tissue samples to be checked under a microscope for signs of disease. - Biopsy: The removal of cell or tissue samples from the urethra, bladder, and, sometimes, the prostate gland. The samples are viewed under a microscope by a pathologist to check for signs of cancer. |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Urethral Cancer ? | Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Where the cancer formed in the urethra. - Whether the cancer has spread through the mucosa lining the urethra to nearby tissue, to lymph nodes, or to other parts of the body. - Whether the patient is a male or female. - The patient's general health. - Whether the cancer has just been diagnosed or has recurred (come back). |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Urethral Cancer ? | Key Points
- After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Urethral cancer is staged and treated based on the part of the urethra that is affected. - Distal urethral cancer - Proximal urethral cancer - Bladder and/or prostate cancer may occur at the same time as urethral cancer.
After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body.
The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan) of the pelvis and abdomen : A procedure that makes a series of detailed pictures of the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the urethra, nearby lymph nodes, and other soft tissue and bones in the pelvis. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Urethrography: A series of x-rays of the urethra. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. A dye is injected through the urethra into the bladder. The dye coats the bladder and urethra and x-rays are taken to see if the urethra is blocked and if cancer has spread to nearby tissue.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer.
Urethral cancer is staged and treated based on the part of the urethra that is affected.
Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal. Distal urethral cancer In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue.
Bladder and/or prostate cancer may occur at the same time as urethral cancer.
In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Urethral Cancer ? | Key Points
- There are different types of treatment for patients with urethral cancer. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Active surveillance - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with urethral cancer.
Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Four types of standard treatment are used:
Surgery Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: - Open excision: Removal of the cancer by surgery. - Transurethral resection (TUR): Surgery to remove the cancer using a special tool inserted into the urethra. - Electroresection with fulguration: Surgery to remove the cancer by electric current. A lighted tool with a small wire loop on the end is used to remove the cancer or to burn the tumor away with high-energy electricity. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove or destroy tissue. - Lymph node dissection: Lymph nodes in the pelvis and groin may be removed. - Cystourethrectomy: Surgery to remove the bladder and the urethra. - Cystoprostatectomy: Surgery to remove the bladder and the prostate. - Anterior exenteration: Surgery to remove the urethra, the bladder, and the vagina. Plastic surgery may be done to rebuild the vagina. - Partial penectomy: Surgery to remove the part of the penis surrounding the urethra where cancer has spread. Plastic surgery may be done to rebuild the penis. - Radical penectomy: Surgery to remove the entire penis. Plastic surgery may be done to rebuild the penis. If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Urethral Cancer
Distal Urethral Cancer
Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: - Surgery to remove the tumor (transurethral resection), electroresection and fulguration, or laser surgery for tumors that have not spread deeply into tissue. - Brachytherapy and/or external radiation therapy for tumors that have not spread deeply into tissue. - Surgery to remove the tumor (anterior exenteration) for tumors that have spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). Radiation therapy may be given before surgery. For men, treatment may include the following: - Surgery to remove the tumor (transurethral resection), electroresection and fulguration, or laser surgery for tumors that have not spread deeply into tissue. - Surgery to remove part of the penis (partial penectomy) for tumors that are near the tip of the penis. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Surgery to remove part of the urethra for tumors that are in the distal urethra but not at the tip of the penis and have not spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Surgery to remove the penis (radical penectomy) for tumors that have spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Radiation therapy with or without chemotherapy. - Chemotherapy given together with radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with distal urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Proximal Urethral Cancer
Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: - Radiation therapy and/or surgery (open excision, transurethral resection) for tumors that are of an inch or smaller. - Radiation therapy followed by surgery (anterior exenteration with lymph node dissection and urinary diversion). For men, treatment may include the following: - Radiation therapy or radiation therapy and chemotherapy, followed by surgery (cystoprostatectomy, penectomy, lymph node dissection, and urinary diversion). Check the list of NCI-supported cancer clinical trials that are now accepting patients with proximal urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Urethral Cancer that Forms with Invasive Bladder Cancer
Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: - Surgery (cystourethrectomy in women, or urethrectomy and cystoprostatectomy in men). If the urethra is not removed during surgery to remove the bladder, treatment may include the following: - Active surveillance. Samples of cells are taken from inside the urethra and checked under a microscope for signs of cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with urethral cancer associated with invasive bladder cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Metastatic or Recurrent Urethral Cancer
Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: - Surgery to remove the tumor. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. |
The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1½ inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. These cancers are named for the types of cells that become malignant (cancer): Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: The following tests and procedures may be used: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal.EnlargeAnatomy of the distal and proximal urethra. Urine flows out of the bladder and leaves the body through the urethra. The part of the urethra that is closest to the bladder is called the proximal urethra. The part that is closest to where the urine leaves the body is called the distal urethra. The urethra is about 8 inches long in men and about 1½ inches long in women. In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about ½ inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later. The cancer may come back in the urethra or in other parts of the body. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. Information about clinical trials is available from the NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: For men, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: If the urethra is not removed during surgery to remove the bladder, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about urethral cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of urethral cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Urethral Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389384] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Urethral Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Urethral Cancer ? | New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. Oral cavity cancer usually forms in the squamous cells (thin, flat cells lining the inside of the oral cavity). Oral cavity cancer forms in any of these tissues of the oral cavity: Pharyngeal cancer forms in the tissues of the pharynx (throat), including the nasopharynx, oropharynx, and hypopharynx. This summary covers nasopharyngeal cancer, which forms in the tissue of the nasopharynx (the upper part of the throat behind the nose). See the following PDQ summaries for more information about the prevention, diagnosis, and treatment of head and neck cancers, including oral cavity cancer and nasopharyngeal cancer: From 2009 to 2018, the number of new cases of oral cavity cancer in the United States slightly increased, mostly in non-Hispanic White patients. Oral cavity cancer is more common in men than in women. Although oral cavity cancer may occur in adults of any age, it occurs most often in those aged 75 to 84 years. France, Brazil, and parts of Asia have much higher rates of oral cavity cancer than most other countries. Nasopharyngeal cancer is rare in the United States. It is more common in parts of Asia, the Arctic region, North Africa, and the Middle East. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor.
Being infected with Epstein-Barr virus (EBV) increases the risk of nasopharyngeal cancer. For more information about risk factors and protective factors for oral cavity cancer, see the PDQ summaries on Oral Cavity, Oropharyngeal, Hypopharyngeal, and Laryngeal Cancers and Lip and Oral Cavity Cancer Treatment (Adult). Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. No studies have shown that screening for oral cavity cancer and nasopharyngeal cancer would lower the risk of dying from these diseases. A dentist or medical doctor may check the oral cavity during a routine check-up. The exam will include looking for lesions, including areas of leukoplakia (an abnormal white patch of cells) and erythroplakia (an abnormal red patch of cells). Leukoplakia and erythroplakia lesions on the mucous membranes may become cancerous.
If lesions are seen in the mouth, the following procedures may be used to find abnormal tissue that might become oral cavity cancer: More than half of oral cancers have already spread to lymph nodes or other areas by the time they are found. Epstein-Barr virus (EBV) has been linked to nasopharyngeal cancer. Screening for nasopharyngeal cancer using the EBV antibody test or EBV DNA test has been studied. These are laboratory tests used to check the blood for EBV antibodies or EBV DNA. If EBV antibodies or DNA are found in the blood more tests may be done to check for nasopharyngeal cancer. No studies have shown that screening would decrease the risk of dying from this disease. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. Some cancers never cause symptoms or become life-threatening, but if found by a screening test, the cancer may be treated. Finding these cancers is called overdiagnosis. It is not known if treatment of oral cavity cancer or nasopharyngeal cancer would help you live longer than if no treatment were given, and treatments for cancer, such as surgery and radiation therapy, may have serious side effects. Screening test results may appear to be normal even though oral cavity cancer or nasopharyngeal cancer is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests and procedures (such as biopsy), which also have risks. A biopsy is needed to diagnose oral cavity and nasopharyngeal cancers. Cells or tissues are removed from the oral cavity or nasopharynx and viewed under a microscope by a pathologist to check for signs of cancer. When the cells are cancer and the pathologist reports them as not being cancer, the cancer is misdiagnosed. Cancer is also misdiagnosed when the cells are not cancer and the pathologist reports there is cancer. When cancer is misdiagnosed, treatment that is needed may not be given or treatment may be given that is not needed. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about oral cavity and nasopharyngeal cancers screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Oral Cavity and Nasopharyngeal Cancers Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389441] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Oral Cavity and Nasopharyngeal Cancers Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Oral Cavity and Oropharyngeal Cancer ? | Key Points
- Oral cavity and oropharyngeal cancer are diseases in which malignant (cancer) cells form in the mouth and throat. - The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender. - Different factors increase or decrease the risk of oral cavity and oropharyngeal cancer.
Oral cavity and oropharyngeal cancer are diseases in which malignant (cancer) cells form in the mouth and throat.
Oral cavity cancer forms in any of these tissues of the oral cavity: - The front two thirds of the tongue. - The gingiva (gums). - The buccal mucosa (the lining of the inside of the cheeks). - The floor (bottom) of the mouth under the tongue. - The hard palate (the front of the roof of the mouth). - The retromolar trigone (the small area behind the wisdom teeth). Oropharyngeal cancer forms in any of these tissues of the oropharynx: - The middle part of the pharynx (throat) behind the mouth. - The back one third of the tongue. - The soft palate (the back of the roof of the mouth). - The side and back walls of the throat. - The tonsils. Most oral cavity and oropharyngeal cancers start in squamous cells, the thin, flat cells that line the lips, oral cavity, and oropharynx. Cancer that forms in squamous cells is called squamous cell carcinoma. See the following PDQ summaries for more information about the screening, diagnosis, and treatment of oral cavity and oropharyngeal cancer: - Oral Cavity and Oropharyngeal Cancer Prevention - Lip and Oral Cavity Cancer Treatment - Oropharyngeal Cancer Treatment
The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender.
Over the past ten years, the number of new cases and deaths from oral cavity and oropharyngeal cancer slightly increased in white men and women. The number slightly decreased among black men and women. Oral cavity and oropharyngeal cancer is more common in men than in women. Although oral cavity and oropharyngeal cancer may occur in adults of any age, it occurs most often in those aged 55 to 64 years. France, Brazil, and parts of Asia have much higher rates of oral cavity and oropharyngeal cancer than most other countries. The number of new cases of oropharyngeal cancer caused by certain types of human papillomavirus (HPV) infection has increased. One kind of HPV, called HPV 16, is often passed from one person to another during sexual activity. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. Oral cavity cancer usually forms in the squamous cells (thin, flat cells lining the inside of the oral cavity). Oral cavity cancer forms in any of these tissues of the oral cavity: Pharyngeal cancer forms in the tissues of the pharynx (throat), including the nasopharynx, oropharynx, and hypopharynx. This summary covers nasopharyngeal cancer, which forms in the tissue of the nasopharynx (the upper part of the throat behind the nose). See the following PDQ summaries for more information about the prevention, diagnosis, and treatment of head and neck cancers, including oral cavity cancer and nasopharyngeal cancer: From 2009 to 2018, the number of new cases of oral cavity cancer in the United States slightly increased, mostly in non-Hispanic White patients. Oral cavity cancer is more common in men than in women. Although oral cavity cancer may occur in adults of any age, it occurs most often in those aged 75 to 84 years. France, Brazil, and parts of Asia have much higher rates of oral cavity cancer than most other countries. Nasopharyngeal cancer is rare in the United States. It is more common in parts of Asia, the Arctic region, North Africa, and the Middle East. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor.
Being infected with Epstein-Barr virus (EBV) increases the risk of nasopharyngeal cancer. For more information about risk factors and protective factors for oral cavity cancer, see the PDQ summaries on Oral Cavity, Oropharyngeal, Hypopharyngeal, and Laryngeal Cancers and Lip and Oral Cavity Cancer Treatment (Adult). Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. No studies have shown that screening for oral cavity cancer and nasopharyngeal cancer would lower the risk of dying from these diseases. A dentist or medical doctor may check the oral cavity during a routine check-up. The exam will include looking for lesions, including areas of leukoplakia (an abnormal white patch of cells) and erythroplakia (an abnormal red patch of cells). Leukoplakia and erythroplakia lesions on the mucous membranes may become cancerous.
If lesions are seen in the mouth, the following procedures may be used to find abnormal tissue that might become oral cavity cancer: More than half of oral cancers have already spread to lymph nodes or other areas by the time they are found. Epstein-Barr virus (EBV) has been linked to nasopharyngeal cancer. Screening for nasopharyngeal cancer using the EBV antibody test or EBV DNA test has been studied. These are laboratory tests used to check the blood for EBV antibodies or EBV DNA. If EBV antibodies or DNA are found in the blood more tests may be done to check for nasopharyngeal cancer. No studies have shown that screening would decrease the risk of dying from this disease. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. Some cancers never cause symptoms or become life-threatening, but if found by a screening test, the cancer may be treated. Finding these cancers is called overdiagnosis. It is not known if treatment of oral cavity cancer or nasopharyngeal cancer would help you live longer than if no treatment were given, and treatments for cancer, such as surgery and radiation therapy, may have serious side effects. Screening test results may appear to be normal even though oral cavity cancer or nasopharyngeal cancer is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests and procedures (such as biopsy), which also have risks. A biopsy is needed to diagnose oral cavity and nasopharyngeal cancers. Cells or tissues are removed from the oral cavity or nasopharynx and viewed under a microscope by a pathologist to check for signs of cancer. When the cells are cancer and the pathologist reports them as not being cancer, the cancer is misdiagnosed. Cancer is also misdiagnosed when the cells are not cancer and the pathologist reports there is cancer. When cancer is misdiagnosed, treatment that is needed may not be given or treatment may be given that is not needed. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about oral cavity and nasopharyngeal cancers screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Oral Cavity and Nasopharyngeal Cancers Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389441] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Oral Cavity and Nasopharyngeal Cancers Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Oral Cavity and Oropharyngeal Cancer? ? | The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender. Over the past ten years, the number of new cases and deaths from oral cavity and oropharyngeal cancer slightly increased in white men and women. The number slightly decreased among black men and women. Oral cavity and oropharyngeal cancer is more common in men than in women. Although oral cavity and oropharyngeal cancer may occur in adults of any age, it occurs most often in those aged 55 to 64 years. France, Brazil, and parts of Asia have much higher rates of oral cavity and oropharyngeal cancer than most other countries. The number of new cases of oropharyngeal cancer caused by certain types of human papillomavirus (HPV) infection has increased. One kind of HPV, called HPV 16, is often passed from one person to another during sexual activity. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. Oral cavity cancer usually forms in the squamous cells (thin, flat cells lining the inside of the oral cavity). Oral cavity cancer forms in any of these tissues of the oral cavity: Pharyngeal cancer forms in the tissues of the pharynx (throat), including the nasopharynx, oropharynx, and hypopharynx. This summary covers nasopharyngeal cancer, which forms in the tissue of the nasopharynx (the upper part of the throat behind the nose). See the following PDQ summaries for more information about the prevention, diagnosis, and treatment of head and neck cancers, including oral cavity cancer and nasopharyngeal cancer: From 2009 to 2018, the number of new cases of oral cavity cancer in the United States slightly increased, mostly in non-Hispanic White patients. Oral cavity cancer is more common in men than in women. Although oral cavity cancer may occur in adults of any age, it occurs most often in those aged 75 to 84 years. France, Brazil, and parts of Asia have much higher rates of oral cavity cancer than most other countries. Nasopharyngeal cancer is rare in the United States. It is more common in parts of Asia, the Arctic region, North Africa, and the Middle East. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor.
Being infected with Epstein-Barr virus (EBV) increases the risk of nasopharyngeal cancer. For more information about risk factors and protective factors for oral cavity cancer, see the PDQ summaries on Oral Cavity, Oropharyngeal, Hypopharyngeal, and Laryngeal Cancers and Lip and Oral Cavity Cancer Treatment (Adult). Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. No studies have shown that screening for oral cavity cancer and nasopharyngeal cancer would lower the risk of dying from these diseases. A dentist or medical doctor may check the oral cavity during a routine check-up. The exam will include looking for lesions, including areas of leukoplakia (an abnormal white patch of cells) and erythroplakia (an abnormal red patch of cells). Leukoplakia and erythroplakia lesions on the mucous membranes may become cancerous.
If lesions are seen in the mouth, the following procedures may be used to find abnormal tissue that might become oral cavity cancer: More than half of oral cancers have already spread to lymph nodes or other areas by the time they are found. Epstein-Barr virus (EBV) has been linked to nasopharyngeal cancer. Screening for nasopharyngeal cancer using the EBV antibody test or EBV DNA test has been studied. These are laboratory tests used to check the blood for EBV antibodies or EBV DNA. If EBV antibodies or DNA are found in the blood more tests may be done to check for nasopharyngeal cancer. No studies have shown that screening would decrease the risk of dying from this disease. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. Some cancers never cause symptoms or become life-threatening, but if found by a screening test, the cancer may be treated. Finding these cancers is called overdiagnosis. It is not known if treatment of oral cavity cancer or nasopharyngeal cancer would help you live longer than if no treatment were given, and treatments for cancer, such as surgery and radiation therapy, may have serious side effects. Screening test results may appear to be normal even though oral cavity cancer or nasopharyngeal cancer is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests and procedures (such as biopsy), which also have risks. A biopsy is needed to diagnose oral cavity and nasopharyngeal cancers. Cells or tissues are removed from the oral cavity or nasopharynx and viewed under a microscope by a pathologist to check for signs of cancer. When the cells are cancer and the pathologist reports them as not being cancer, the cancer is misdiagnosed. Cancer is also misdiagnosed when the cells are not cancer and the pathologist reports there is cancer. When cancer is misdiagnosed, treatment that is needed may not be given or treatment may be given that is not needed. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about oral cavity and nasopharyngeal cancers screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Oral Cavity and Nasopharyngeal Cancers Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389441] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Oral Cavity and Nasopharyngeal Cancers Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Oral Cavity and Oropharyngeal Cancer? ? | Different factors increase or decrease the risk of oral cavity and oropharyngeal cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for oral cavity and oropharyngeal cancer, see the PDQ summary on Oral Cavity and Oropharyngeal Cancer Prevention. |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied, including: The anus is the end of the large intestine, below the rectum, through which stool (solid waste) leaves the body. The anus is formed partly from the outer skin layers of the body and partly from the intestine. Two ring-like muscles, called sphincter muscles, open and close the anal opening and let stool pass out of the body. The anal canal, the part of the anus between the rectum and the anal opening, is about 1-1½ inches long. EnlargeAnatomy of the lower gastrointestinal (digestive) system showing the colon, rectum, and anus. Other organs that make up the digestive system are also shown. The skin around the outside of the anus is called the perianal area. Tumors in this area are skin tumors, not anal cancer. See the following PDQ summary for more information about anal cancer: In the United States, the most common type of anal cancer is squamous cell carcinoma. Studies show that human papillomavirus (HPV) infection is the main cause of this type of anal cancer. Another type of anal cancer, called anal adenocarcinoma, is very rare and is not discussed in this summary. From 2007 to 2016, new cases of anal cancer and deaths from anal cancer increased each year. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Being infected with human papillomavirus (HPV) is the main risk factor for anal cancer. Being infected with HPV can lead to squamous cell carcinoma of the anus, the most common type of anal cancer. About nine out of every ten cases of anal cancer are found in patients with anal HPV infection. Patients with healthy immune systems are usually able to fight HPV infections. Patients with weakened immune systems who are infected with HPV have a higher risk of anal cancer. Cervical cancer, vaginal cancer, and vulvar cancer are related to HPV infection. Women who have had cervical, vaginal, or vulvar cancer have a higher risk of anal cancer. Being infected with human immunodeficiency virus (HIV) is a strong risk factor for anal cancer. HIV is the cause of acquired immunodeficiency syndrome (AIDS). HIV weakens the body's immune system and its ability to fight infection. HPV infection of the anus is common among patients who are HIV-positive. The risk of anal cancer is higher in men who are HIV-positive and have sex with men compared with men who are HIV-negative and have sex with men. Women who are HIV-positive also have an increased risk of anal cancer compared with women who are HIV-negative. Studies show that intravenous drug use or cigarette smoking may further increase the risk of anal cancer in patients who are HIV-positive. Immunosuppression is a condition that weakens the body's immune system and its ability to fight infections and other diseases. Chronic (long-term) immunosuppression may increase the risk of anal cancer because it lowers the body's ability to fight HPV infection. Patients who have an organ transplant and receive immunosuppressive medicine to prevent organ rejection have an increased risk of anal cancer. Having an autoimmune disorder such as Crohn disease or psoriasis may increase the risk of anal cancer. It is not clear if the increased risk is due to the autoimmune condition, the treatment for the condition, or a combination of both. The following sexual practices increase the risk of anal cancer because they increase the chance of being infected with HPV: Men and women who have a history of anal warts or other sexually transmitted diseases also have an increased risk of anal cancer. Studies show that cigarette smoking increases the risk of anal cancer. Studies also show that current smokers have a higher risk of anal cancer than smokers who have quit or people who have never smoked. The human papillomavirus (HPV) vaccine is used to prevent anal cancer, cervical cancer, vulvar cancer, and vaginal cancer caused by HPV. It is also used to prevent lesions caused by HPV that may become cancer in the future. Studies show that being vaccinated against HPV lowers the risk of anal cancer. The vaccine may work best when it is given before a person is exposed to HPV. It is not known if the use of condoms protects against anal HPV infection. This is because not enough studies have been done to prove this. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about anal cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Anal Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/anal/patient/anal-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389512] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Anal Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to prevent Anal Cancer ? | Key Points
- Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for anal cancer: - Anal HPV infection - Certain medical conditions - History of cervical, vaginal, or vulvar cancer - HIV infection/AIDS - Immunosuppression - Certain sexual practices - Cigarette smoking - The following protective factor decreases the risk of anal cancer: - HPV vaccine - It is not clear if the following protective factor decreases the risk of anal cancer: - Condom use - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent anal cancer are being studied in clinical trials.
Avoiding risk factors and increasing protective factors may help prevent cancer.
Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer.
The following are risk factors for anal cancer:
Anal HPV infection Being infected with human papillomavirus (HPV) is the main risk factor for anal cancer. Being infected with HPV can lead to squamous cell carcinoma of the anus, the most common type of anal cancer. About nine out of every ten cases of anal cancer are found in patients with anal HPV infection. Patients with healthy immune systems are usually able to fight HPV infections. Patients with weakened immune systems who are infected with HPV have a higher risk of anal cancer. Certain medical conditions History of cervical, vaginal, or vulvar cancer Cervical cancer, vaginal cancer, and vulvar cancer are related to HPV infection. Women who have had cervical, vaginal, or vulvar cancer have a higher risk of anal cancer. HIV infection/AIDS Being infected with human immunodeficiency virus (HIV) is a strong risk factor for anal cancer. HIV is the cause of acquired immunodeficiency syndrome (AIDS). HIV weakens the body's immune system and its ability to fight infection. HPV infection of the anus is common among patients who are HIV-positive. The risk of anal cancer is higher in men who are HIV-positive and have sex with men compared with men who are HIV-negative and have sex with men. Women who are HIV-positive also have an increased risk of anal cancer compared with women who are HIV-negative. Studies show that intravenous drug use or cigarette smoking may further increase the risk of anal cancer in patients who are HIV-positive. Immunosuppression Immunosuppression is a condition that weakens the body's immune system and its ability to fight infections and other diseases. Chronic (long-term) immunosuppression may increase the risk of anal cancer because it lowers the body's ability to fight HPV infection. Patients who have an organ transplant and receive immunosuppressive medicine to prevent organ rejection have an increased risk of anal cancer. Having an autoimmune disorder such as Crohn disease or psoriasis may increase the risk of anal cancer. It is not clear if the increased risk is due to the autoimmune condition, the treatment for the condition, or a combination of both. Certain sexual practices The following sexual practices increase the risk of anal cancer because they increase the chance of being infected with HPV: - Having receptive anal intercourse (anal sex). - Having many sexual partners. - Sex between men. Men and women who have a history of anal warts or other sexually transmitted diseases also have an increased risk of anal cancer. Cigarette smoking Studies show that cigarette smoking increases the risk of anal cancer. Studies also show that current smokers have a higher risk of anal cancer than smokers who have quit or people who have never smoked.
The following protective factor decreases the risk of anal cancer:
HPV vaccine The human papillomavirus (HPV) vaccine is used to prevent anal cancer, cervical cancer, vulvar cancer, and vaginal cancer caused by HPV. It is also used to prevent lesions caused by HPV that may become cancer in the future. Studies show that being vaccinated against HPV lowers the risk of anal cancer. The vaccine may work best when it is given before a person is exposed to HPV.
It is not clear if the following protective factor decreases the risk of anal cancer:
Condom use It is not known if the use of condoms protects against anal HPV infection. This is because not enough studies have been done to prove this.
Cancer prevention clinical trials are used to study ways to prevent cancer.
Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements.
New ways to prevent anal cancer are being studied in clinical trials.
Clinical trials are taking place in many parts of the country. Information about clinical trials for anal cancer prevention can be found in the Clinical Trials section of the NCI Web site. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms.
If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. Although there is no standard or routine screening tests for liver cancer, the following tests are being used or studied to screen for it: Ultrasound is a procedure in which high-energy waves (ultrasound) are bounced off the liver and make echoes. The echoes form a picture of the liver called a sonogram. CT scan is a procedure that makes a series of detailed pictures of the liver, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the liver show up more clearly. This procedure is also called CAT scan or computed tomography. Tumor markers, also called biomarkers, are substances made by the tumor that may be found in the blood, other body fluids, or tissues. A high level of a specific tumor marker may mean that a certain type of cancer is present in the body. Alpha-fetoprotein (AFP) is the most widely used tumor marker for detecting liver cancer. However, other cancers and certain conditions, including pregnancy, hepatitis, and other types of cancer, may also increase AFP levels. Specific tumor markers that may lead to early detection of liver cancer are being studied. Doctors use these screening tests to find, or diagnose, liver cancer. Learn more about Liver Cancer Diagnosis. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risk of harms associated with them. Before having any screening test, you may want to discuss the test with your doctor. It is important to be aware of any possible harms of the test and whether the test has been proven to reduce the risk of dying from cancer. The risks of liver cancer screening include the following: Your doctor can advise you about your risk for liver cancer and your need for screening tests. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Liver Cancer Screening was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Liver (Hepatocellular) Cancer ? | Key Points
- Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - Liver cancer is less common in the United States than in other parts of the world. - Having hepatitis or cirrhosis can increase the risk of developing liver cancer.
Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver.
The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fats from food. - To store glycogen (sugar), which the body uses for energy. See the following PDQ summaries for more information about liver (hepatocellular) cancer: - Liver (Hepatocellular) Cancer Prevention - Adult Primary Liver Cancer Treatment - Childhood Liver Cancer Treatment
Liver cancer is less common in the United States than in other parts of the world.
Liver cancer is uncommon in the United States, but is the fourth most common cancer in the world. In the United States, men, especially Chinese American men, have a greater risk of developing liver cancer. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms.
If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. Although there is no standard or routine screening tests for liver cancer, the following tests are being used or studied to screen for it: Ultrasound is a procedure in which high-energy waves (ultrasound) are bounced off the liver and make echoes. The echoes form a picture of the liver called a sonogram. CT scan is a procedure that makes a series of detailed pictures of the liver, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the liver show up more clearly. This procedure is also called CAT scan or computed tomography. Tumor markers, also called biomarkers, are substances made by the tumor that may be found in the blood, other body fluids, or tissues. A high level of a specific tumor marker may mean that a certain type of cancer is present in the body. Alpha-fetoprotein (AFP) is the most widely used tumor marker for detecting liver cancer. However, other cancers and certain conditions, including pregnancy, hepatitis, and other types of cancer, may also increase AFP levels. Specific tumor markers that may lead to early detection of liver cancer are being studied. Doctors use these screening tests to find, or diagnose, liver cancer. Learn more about Liver Cancer Diagnosis. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risk of harms associated with them. Before having any screening test, you may want to discuss the test with your doctor. It is important to be aware of any possible harms of the test and whether the test has been proven to reduce the risk of dying from cancer. The risks of liver cancer screening include the following: Your doctor can advise you about your risk for liver cancer and your need for screening tests. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Liver Cancer Screening was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Liver (Hepatocellular) Cancer? ? | Having hepatitis or cirrhosis can increase the risk of developing liver cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for liver cancer include: - Having hepatitis B or hepatitis C; having both hepatitis B and hepatitis C increases the risk even more. - Having cirrhosis, which can be caused by: - hepatitis (especially hepatitis C); or - drinking large amounts of alcohol for many years or being an alcoholic. - Eating foods tainted with aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly). |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Childhood Soft Tissue Sarcoma ? | Key Points
- Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body. - Soft tissue sarcoma occurs in children and adults. - Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma. - The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. - Diagnostic tests are used to detect (find) and diagnose childhood soft tissue sarcoma. - If tests show there may be a soft tissue sarcoma, a biopsy is done. - There are many different types of soft tissue sarcomas. - Fat tissue tumors - Bone and cartilage tumors - Fibrous (connective) tissue tumors - Skeletal muscle tumors - Smooth muscle tumors - So-called fibrohistiocytic tumors - Peripheral nervous system tumors - Pericytic (Perivascular) Tumors - Tumors of unknown origin - Blood vessel tumors - Certain factors affect prognosis (chance of recovery) and treatment options.
Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following: - Fat. - A mix of bone and cartilage. - Fibrous tissue. - Muscles. - Nerves. - Tendons (bands of tissue that connect muscles to bones). - Synovial tissues (tissues around joints). - Blood vessels. - Lymph vessels. Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).
Soft tissue sarcoma occurs in children and adults.
Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for information on treatment in adults.)
There are many different types of soft tissue sarcomas.
The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors - Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma. Myxoid liposarcoma is usually low grade and responds well to treatment. The cells of myxoid liposarcoma have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose myxoid liposarcoma, the tumor cells are checked for this genetic change. Pleomorphic liposarcoma is usually high grade (likely to grow and spread quickly) and is less likely to respond well to treatment. Bone and cartilage tumors Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: - Extraskeletal mesenchymal chondrosarcoma . This type of bone and cartilage tumor often affects young adults and occurs in the head and neck. - Extraskeletal osteosarcoma . This type of bone and cartilage tumor is very rare in children and adolescents. It is likely to come back after treatment and may spread to the lungs. Fibrous (connective) tissue tumors Fibrous (connective) tissue tumors include the following types: - Desmoid-type fibromatosis (also called desmoid tumor or aggressive fibromatosis). This fibrous tissue tumor is low grade (likely to grow slowly). It may come back in nearby tissues but usually does not spread to distant parts of the body. Rarely, the tumor may disappear without treatment. Desmoid tumors sometimes occur in children with changes in the adenomatous polyposis coli (APC) gene. Changes in this gene cause familial adenomatous polyposis (FAP). FAP is an inherited condition in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed. - Dermatofibrosarcoma protuberans . This is a rare tumor of the deep layers of the skin found in children and adults. The cells of this tumor have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose dermatofibrosarcoma protuberans, the tumor cells are checked for this genetic change. - Fibrosarcoma . There are two types of fibrosarcoma in children and adolescents: - Infantile fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma is found in children aged 4 years and younger. It most often occurs in infants and may be seen in a prenatal ultrasound exam. This tumor is often large and fast growing, but rarely spreads to distant parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose infantile fibrosarcoma, the tumor cells are checked for this genetic change. - Adult-type fibrosarcoma. This is the same type of fibrosarcoma found in adults. The cells of this tumor do not have the genetic change found in infantile fibrosarcoma. See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information. - Inflammatory myofibroblastic tumor . This is a fibrous tissue tumor that occurs in children and adolescents. It is likely to come back after treatment but rarely spreads to distant parts of the body. A certain genetic change has been found in about half of these tumors. - Low-grade fibromyxoid sarcoma . This is a slow-growing tumor that affects young and middle-aged adults. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose low-grade fibromyxoid sarcoma, the tumor cells are checked for this genetic change. The tumor may come back many years after treatment and spread to the lungs and the lining of the wall of the chest cavity. Lifelong follow-up is needed. - Myxofibrosarcoma . This is a rare fibrous tissue tumor that is found less often in children than in adults. - Sclerosing epithelioid fibrosarcoma . This is a rare fibrous tissue tumor that can come back and spread to other places years after treatment. Long-term follow-up is needed. Skeletal muscle tumors Skeletal muscle is attached to bones and helps the body move. - Rhabdomyosarcoma . Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in children 14 years and younger. See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information. Smooth muscle tumors Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. - Leiomyosarcoma . This smooth muscle tumor has been linked with Epstein-Barr virus in children who also have HIV disease or AIDS. Leiomyosarcoma may also form as a second cancer in survivors of inherited retinoblastoma, sometimes many years after the initial treatment for retinoblastoma. So-called fibrohistiocytic tumors - Plexiform fibrohistiocytic tumor . This is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just under the skin on the arm, hand, or wrist. It may rarely spread to nearby lymph nodes or to the lungs. Peripheral nervous system tumors Peripheral nervous system tumors include the following types: - Ectomesenchymoma . This is a rare, fast-growing tumor of the nerve sheath (protective covering of nerves that are not part of the brain or spinal cord) that occurs mainly in children. Ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, arms, or legs. - Malignant peripheral nerve sheath tumor . This is a tumor that forms in the nerve sheath. Some children who have a malignant peripheral nerve sheath tumor have a rare genetic condition called neurofibromatosis type 1 (NF1). This tumor may be low grade or high grade. - Malignant triton tumor . These are very rare, fast-growing tumors that occur most often in children with NF1. Pericytic (Perivascular) Tumors Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: - Myopericytoma . Infantile hemangiopericytoma is a type of myopericytoma. Children younger than 1 year at the time of diagnosis may have a better prognosis. In patients older than 1 year, infantile hemangiopericytoma is more likely to spread to other parts of the body, including the lymph nodes and lungs. - Infantile myofibromatosis . Infantile myofibromatosis is another type of myopericytoma. It is a fibrous tumor that often forms in the first 2 years of life. There may be one nodule under the skin, usually in the head and neck area (myofibroma), or nodules in several skin areas, muscle, and bone (myofibromatosis). These tumors may go away without treatment. Tumors of unknown origin Tumors of unknown origin (the place where the tumor first formed is not known) include the following types: - Alveolar soft part sarcoma . This is a rare tumor of the soft supporting tissue that connects and surrounds the organs and other tissues. It is most commonly found in the limbs but can occur in the tissues of the mouth, jaws, and face. It may grow slowly and may have spread to other parts of the body at the time of diagnosis. Alveolar soft part sarcoma may have a better prognosis when the tumor is 5 centimeters or smaller or when the tumor is completely removed by surgery. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose alveolar soft part sarcoma, the tumor cells are checked for this genetic change. - Clear cell sarcoma of soft tissue . This is a slow-growing soft tissue tumor that begins in a tendon (tough, fibrous, cord-like tissue that connects muscle to bone or to another part of the body). Clear cell sarcoma most commonly occurs in deep tissue of the foot, heel, and ankle. It may spread to nearby lymph nodes. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose clear cell sarcoma of soft tissue, the tumor cells are checked for this genetic change. - Desmoplastic small round cell tumor . This tumor most often forms in the abdomen, pelvis or tissues around the testes, but it may form in the kidney. Desmoplastic small round cell tumor may also spread to the lungs and other parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose desmoplastic small round cell tumor, the tumor cells are checked for this genetic change. - Epithelioid sarcoma . This is a rare sarcoma that usually starts deep in soft tissue as a slow growing, firm lump and may spread to the lymph nodes. - Extrarenal (extracranial) rhabdoid tumor . This is a rare, fast-growing tumor of soft tissues such as the liver and peritoneum. It usually occurs in young children, including newborns, but it can occur in older children and adults. Rhabdoid tumors may be linked to a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth. Changes in the SMARCB1 gene may be inherited (passed on from parents to offspring). Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed. - Extraskeletal myxoid chondrosarcoma . This is a rare soft tissue sarcoma that may be found in children and adolescents. Over time, it tends to spread to other parts of the body, including the lymph nodes and lungs. The cells of this tumor usually have a genetic change, often a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose extraskeletal myxoid chondrosarcoma, the tumor cells are checked for this genetic change. The tumor may come back many years after treatment. - Perivascular epithelioid cell tumors (PEComas). Benign (not cancer) PEComas may be found in children with an inherited condition called tuberous sclerosis. They occur in the stomach, intestines, lungs, female reproductive organs, and genitourinary organs. - Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor . See the PDQ summary on Ewing Sarcoma Treatment for information. - Synovial sarcoma . Synovial sarcoma is a common type of soft tissue sarcoma in children and adolescents. Synovial sarcoma usually forms in the tissues around the joints in the arms or legs, but may also form in the trunk, head, or neck. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). Larger tumors have a greater risk of spreading to other parts of the body, including the lungs. Children younger than 10 years and those whose tumor is 5 centimeters or smaller have a better prognosis. - Undifferentiated /unclassified sarcoma . These tumors usually occur in the muscles that are attached to bones and that help the body move. - Undifferentiated pleomorphic sarcoma /malignant fibrous histiocytoma (high-grade). This type of soft tissue tumor may form in parts of the body where patients have received radiation therapy in the past, or as a second cancer in children with retinoblastoma. The tumor is usually found on the arms or legs and may spread to other parts of the body. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for information about malignant fibrous histiocytoma of bone. Blood vessel tumors Blood vessel tumors include the following types: - Angiosarcoma of the soft tissue. Angiosarcoma of the soft tissue is a fast-growing tumor that forms in blood vessels or lymph vessels in any part of the body. Most angiosarcomas are in or just under the skin. Those in deeper soft tissue can form in the liver, spleen, and lung. They are very rare in children, who sometimes have more than one tumor in the skin or liver. Rarely, infantile hemangioma may become angiosarcoma of the soft tissue. (See the PDQ summary on Childhood Vascular Tumors Treatment for more information.) - Epithelioid hemangioendothelioma. Epithelioid hemangioendotheliomas can occur in children, but are most common in adults between 30 and 50 years of age. They usually occur in the liver, lung, or bone. They may be either fast growing or slow growing. In about a third of cases, the tumor spreads to other parts of the body very quickly. (See the PDQ summary on Childhood Vascular Tumors Treatment for more information.) See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: - Childhood Rhabdomyosarcoma Treatment. - Ewing Sarcoma Treatment. - Unusual Cancers of Childhood Treatment (gastrointestinal stromal tumors). |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Childhood Soft Tissue Sarcoma? ? | Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: - Li-Fraumeni syndrome. - Familial adenomatous polyposis (FAP). - Retinoblastoma 1 gene changes. - Neurofibromatosis type 1 (NF1). - Werner syndrome. Other risk factors include the following: - Past treatment with radiation therapy. - Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection at the same time. |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Childhood Soft Tissue Sarcoma ? | The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your childs doctor if your child has any of these problems. |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Childhood Soft Tissue Sarcoma ? | Diagnostic tests are used to detect (find) and diagnose childhood soft tissue sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - X-rays : An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
- If tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies is usually used: - Core needle biopsy : The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI. - Incisional biopsy : The removal of part of a lump or a sample of tissue. - Excisional biopsy : The removal of an entire lump or area of tissue that doesnt look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin. This type of biopsy is rarely used because cancer cells may remain after the biopsy. If cancer cells remain, the cancer may come back or it may spread to other parts of the body. An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor is and may be used to guide future surgery or radiation therapy. The placement of needles or incisions for the biopsy can affect the success of later surgery to remove the tumor. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. In order to plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: - Molecular test : A laboratory test to check for certain genes, proteins, or other molecules in a sample of tissue, blood, or other body fluid. A molecular test may be done with other procedures, such as biopsies, to help diagnose some types of cancer. Molecular tests check for certain gene or chromosome changes that occur in some soft tissue sarcomas. - Reverse transcriptionpolymerase chain reaction (RTPCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for changes in the expression of certain genes. When genes are expressed they make specific proteins that are needed for the structure, function, and monitoring of the bodys tissues and organs. This test is done in order to identify the type of tumor. - Cytogenetic analysis : A laboratory test in which cells in a sample of bone marrow, blood, amniotic fluid, tumor or other tissue is viewed under a microscope to look for changes in the chromosomes. Fluorescence in situ hybridization (FISH) is a type of cytogenetic analysis. - Immunocytochemistry : A test that uses antibodies to check for certain antigens (markers) in a sample of cells. The antibody is usually linked to an enzyme or fluorescent dye that causes the cells that have that marker to become visible under a microscope. This type of test may be used to tell the difference between different types of soft tissue sarcoma. |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Childhood Soft Tissue Sarcoma ? | Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The part of the body where the tumor first formed. - The size and grade of the tumor. - The type of soft tissue sarcoma. - How deep the tumor is under the skin. - Whether the tumor has spread to other places in the body. - The amount of tumor remaining after surgery to remove it. - Whether radiation therapy was used to treat the tumor. - The age and gender of the patient. - Whether the cancer has just been diagnosed or has recurred (come back). |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Childhood Soft Tissue Sarcoma ? | Key Points
- After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body.
After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. In order to plan treatment, it is important to know the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: - Sentinel lymph node biopsy: A sentinel lymph node biopsy is done to check if cancer has spread to the lymph nodes. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A small amount of a radioactive substance and/or blue dye is injected near the tumor. The radioactive substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. This procedure is used for epithelioid and clear cell sarcoma. - PET scan: A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This procedure is also called positron emission tomography (PET) scan. - PET-CT scan: A procedure that combines the pictures from a PET scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Childhood Soft Tissue Sarcoma ? | New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissue include the following: Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.EnlargeSoft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Soft Tissue Sarcoma for information on treatment in adults.) Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: Other risk factors include the following: A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems. The following tests and procedures may be used: The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used: An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor formed and may be used to guide future surgery or radiation therapy. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery. To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including: Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: Fibrous (connective) tissue tumors include the following types: Desmoid tumors sometimes occur in children with changes in the APC gene. Changes in this gene may also cause familial adenomatous polyposis (FAP). FAP is an inherited condition (passed on from parents to offspring) in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and options for gene testing) may be needed. There are two types of fibrosarcoma in children and adolescents: Skeletal muscle is attached to bones and helps the body move. Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types: Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) include the following types: Blood vessel tumors include the following types: See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. To plan treatment, it is important to know
the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment. Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy. The following types of surgery may be used: A second surgery may be needed to: If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. New types of tyrosine kinase inhibitors are being studied such as: Other types of targeted therapy are being studied in clinical trials, including the following: See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Interferon and immune checkpoint inhibitor therapy are types of immunotherapy. There are two types of immune checkpoint inhibitor therapy: Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma. Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. This summary section describes treatments that are being
studied in
clinical trials.
It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of newly diagnosed liposarcoma may include the following: Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following: Treatment of newly diagnosed extraskeletal osteosarcoma may include the following: See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma. Treatment of newly diagnosed desmoid-type fibromatosis may include the following: Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following: Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following: Treatment of newly diagnosed infantile fibrosarcoma may include the following: Treatment of adult newly diagnosed fibrosarcoma may include the following: Treatment of newly diagnosed myxofibrosarcoma may include the following: Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following: Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following: See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Treatment of newly diagnosed leiomyosarcoma may include the following: Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following: Treatment of newly diagnosed malignant peripheral nerve sheath tumor may include the following: It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment. Treatment of newly diagnosed ectomesenchymoma may include the following: Treatment of newly diagnosed infantile hemangiopericytoma may include the following: Treatment of newly diagnosed infantile myofibromatosis may include the following: Treatment of newly diagnosed synovial sarcoma may include the following: Treatment of newly diagnosed epithelioid sarcoma may include the following: Treatment of newly diagnosed alveolar soft part sarcoma may include the following: Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following: Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following: See the PDQ summary on Ewing Sarcoma Treatment. There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following: Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following: Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following: There is no standard treatment for these tumors. See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following: Treatment of newly diagnosed angiosarcoma may include the following: Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following: For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Childhood Soft Tissue Sarcoma ? | Key Points
- There are different types of treatment for patients with childhood soft tissue sarcoma. - Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for childhood soft tissue sarcoma may cause side effects. - Eight types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Observation - Hormone therapy - Nonsteroidal anti-inflammatory drugs - Targeted therapy - Immunotherapy - New types of treatment are being tested in clinical trials. - Gene therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with childhood soft tissue sarcoma.
Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: - Pediatrician. - Radiation oncologist. - Pediatric hematologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. - Child-life specialist.
Treatment for childhood soft tissue sarcoma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)
Eight types of standard treatment are used:
Surgery Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant therapy. The following types of surgery may be used: - Wide local excision: Removal of the tumor along with some normal tissue around it. - Amputation: Surgery to remove all or part of the limb or appendage with cancer, such as the arm or hand. - Lymphadenectomy: Removal of the lymph nodes with cancer. - Mohs surgery: A surgical procedure used to treat cancer in the skin. Individual layers of cancer tissue are removed and checked under a microscope one at a time until all cancer tissue has been removed. This type of surgery is used to treat dermatofibrosarcoma protuberans. It is also called Mohs micrographic surgery. - Hepatectomy: Surgery to remove all or part of the liver. A second surgery may be needed to: - Remove any remaining cancer cells. - Check the area around where the tumor was removed for cancer cells and then remove more tissue if needed. If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether the tumor was completely removed by surgery. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: - Complete removal of the tumor is not possible. - No other treatments are available. - The tumor is not likely to damage any vital organs. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Nonsteroidal anti-inflammatory drugs Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do. Kinase inhibitors are a type of targeted therapy that block an enzyme called kinase (a type of protein). There are different types of kinases in the body that have different actions. - ALK inhibitors may stop the cancer from growing and spreading: - Crizotinib may be used to treat inflammatory myofibroblastic tumor. - Tyrosine kinase inhibitors (TKIs) block signals needed for tumors to grow: - Imatinib is used to treat dermatofibrosarcoma protuberans. - Pazopanib may be used to treat recurrent and progressive soft tissue sarcoma. It is being studied for many types of newly diagnosed soft tissue sarcoma. - Sorafenib may be used to treat desmoid-type fibromatosis. New types of tyrosine kinase inhibitors are being studied such as LOXO-101 and entrectinib for infantile fibrosarcoma. Other types of targeted therapy are being studied in clinical trials, including the following: - mTOR inhibitors are a type of targeted therapy that stops the protein that helps cells divide and survive. mTOR inhibitors are being studied to treat perivascular epithelioid cell tumors (PEComas) and epithelioid hemangioendothelioma. Sirolimus is a type of mTOR inhibitor therapy. - Angiogenesis inhibitors are a type of targeted therapy that prevent the growth of new blood vessels needed for tumors to grow. Angiogenesis inhibitors, such as cediranib, sunitinib, and thalidomide are being studied to treat alveolar soft part sarcoma and epithelioid hemangioendothelioma. Bevacizumab is being studied for blood vessel tumors. - Histone methyltransferase (HMT) inhibitors are targeted therapy drugs that work inside cancer cells and block signals needed for tumors to grow. HMT inhibitors are being studied for the treatment of epithelioid sarcoma, malignant peripheral nerve sheath tumor, extrarenal (extracranial) rhabdoid tumor, extraskeletal myxoid chondrosarcoma, and synovial sarcoma. - Heat-shock protein inhibitors block certain proteins that protect tumor cells and help them grow. Ganetespib is a heat shock protein inhibitor being studied in combination with the mTOR inhibitor sirolimus for malignant peripheral nerve sheath tumors that cannot be removed by surgery. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied for the treatment of rhabdomyosarcoma, malignant peripheral nerve sheath tumor, and synovial sarcoma. See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight disease. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against disease. Interferon is a type of immunotherapy used to treat epithelioid hemangioendothelioma. It interferes with the division of tumor cells and can slow tumor growth.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Soft Tissue Sarcoma
Newly Diagnosed Childhood Soft Tissue Sarcoma
Fat Tissue Tumors Liposarcoma Treatment of liposarcoma may include the following: - Surgery to completely remove the tumor. If the cancer is not completely removed, a second surgery may be done. - Chemotherapy to shrink the tumor, followed by surgery. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Bone and Cartilage Tumors Extraskeletal mesenchymal chondrosarcoma Treatment of extraskeletal mesenchymal chondrosarcoma may include the following: - Surgery to completely remove the tumor. Radiation therapy may be given before and/or after surgery. - Chemotherapy followed by surgery. Chemotherapy with or without radiation therapy is given after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Extraskeletal osteosarcoma Treatment of extraskeletal osteosarcoma may include the following: - Surgery to completely remove the tumor, followed by chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information. Fibrous (Connective) Tissue Tumors Desmoid-type fibromatosis Treatment of desmoid-type fibromatosis may include the following: - Surgery to completely remove the tumor. Treatment before surgery may include the following: - Observation. - Chemotherapy. - Radiation therapy. - Antiestrogen drug therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. If the tumor is not completely removed by surgery, treatment may include the following: - Observation, if other treatment options are not possible. - Radiation therapy. - Radiation therapy or chemotherapy for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Treatment of desmoid-type fibromatosis that has come back may include the following: - Observation and possibly surgery at a later time. - Chemotherapy. Dermatofibrosarcoma protuberans Treatment of dermatofibrosarcoma protuberans may include the following: - Surgery to completely remove the tumor when possible. This may include Mohs surgery. - Radiation therapy before or after surgery. - Targeted therapy (imatinib) if the tumor cannot be removed or has come back. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Fibrosarcoma Infantile fibrosarcoma Treatment of infantile fibrosarcoma may include the following: - Surgery to remove the tumor when possible, followed by observation. - Surgery followed by chemotherapy. - Chemotherapy to shrink the tumor, followed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (tyrosine kinase inhibitor). Adult-type fibrosarcoma Treatment of adult-type fibrosarcoma may include the following: - Surgery to completely remove the tumor when possible. Inflammatory myofibroblastic tumor Treatment of inflammatory myofibroblastic tumor may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy. - Steroid therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. - Targeted therapy (ALK inhibitors). - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Low-grade fibromyxoid sarcoma Treatment of low-grade fibromyxoid sarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Myxofibrosarcoma Treatment of myxofibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Sclerosing epithelioid fibrosarcoma Treatment of sclerosing epithelioid fibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Skeletal Muscle Tumors Rhabdomyosarcoma See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Smooth Muscle Tumors Leiomyosarcoma Treatment of leiomyosarcoma may include the following: - Chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. So-called Fibrohistiocytic Tumors Plexiform fibrohistiocytic tumor Treatment of plexiform fibrohistiocytic tumor may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Peripheral Nervous System Tumors Ectomesenchymoma Treatment of ectomesenchymoma may include the following: - Surgery and chemotherapy. - Radiation therapy. Malignant peripheral nerve sheath tumor Treatment of malignant peripheral nerve sheath tumor may include the following: - Surgery to completely remove the tumor when possible. - Radiation therapy before or after surgery. - Chemotherapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Malignant triton tumor Malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. A regimen of targeted therapy, radiation therapy, and surgery with or without chemotherapy is being studied. Pericytic (Perivascular) Tumors Infantile hemangiopericytoma Treatment of infantile hemangiopericytoma may include the following: - Chemotherapy. Infantile myofibromatosis Treatment of infantile myofibromatosis may include the following: - Combination chemotherapy. Tumors of Unknown Origin (the place where the tumor first formed is not known) Alveolar soft part sarcoma Treatment of alveolar soft part sarcoma may include the following: - Surgery to completely remove the tumor when possible. - Radiation therapy before or after surgery, if the tumor cannot be completely removed by surgery. - Targeted therapy (angiogenesis inhibitor). - A clinical trial of targeted therapy (angiogenesis inhibitor) for children. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Clear cell sarcoma of soft tissue Treatment of clear cell sarcoma of soft tissue may include the following: - Surgery to remove the tumor when possible. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Desmoplastic small round cell tumor There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy followed by surgery. - Radiation therapy. Epithelioid sarcoma Treatment of epithelioid sarcoma may include the following: - Surgery to remove the tumor when possible. - Chemotherapy before or after surgery. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Extrarenal (extracranial) rhabdoid tumor Treatment of extrarenal (extracranial) rhabdoid tumor may include the following: - A combination of surgery to remove the tumor when possible, chemotherapy, and radiation therapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Extraskeletal myxoid chondrosarcoma Treatment of extraskeletal myxoid chondrosarcoma may include the following: - Surgery to remove the tumor when possible. - Radiation therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Perivascular epithelioid cell tumors (PEComas) Treatment of perivascular epithelioid cell tumors may include the following: - Surgery to remove the tumor. - Observation followed by surgery. - Targeted therapy (mTOR inhibitor), for tumors that have certain gene changes and cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor See the PDQ summary on Ewing Sarcoma Treatment. Synovial sarcoma Treatment of synovial sarcoma may include the following: - Chemotherapy. - Surgery. Radiation therapy and/or chemotherapy may be given before or after surgery. - A clinical trial of gene therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. Undifferentiated/unclassified sarcoma These tumors include undifferentiated pleomorphic sarcoma /malignant fibrous histiocytoma (high-grade). There is no standard treatment for these tumors. Treatment may include the following: - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Blood Vessel Tumors Angiosarcoma of soft tissue Treatment of angiosarcoma may include the following: - Surgery to completely remove the tumor. - A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. - Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Epithelioid hemangioendothelioma Treatment of epithelioid hemangioendothelioma may include the following: - Surgery to remove the tumor when possible. - Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread. - Chemotherapy. - Total hepatectomy and liver transplant when the tumor is in the liver. Metastatic Childhood Soft Tissue Sarcoma Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: - Chemotherapy and radiation therapy. Surgery may be done to remove tumors that have spread to the lung. - Stereotactic body radiation therapy for tumors that have spread to the lung. For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Check the list of NCI-supported cancer clinical trials that are now accepting patients with nonmetastatic childhood soft tissue sarcoma and metastatic childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following: - Surgery to remove cancer that has come back where it first formed or that has spread to the lung. - Surgery followed by external or internal radiation therapy, if radiation therapy has not already been given. - Surgery to remove the arm or leg with cancer, if radiation therapy was already given. - Chemotherapy. - Targeted therapy (tyrosine kinase inhibitor). - A clinical trial of a new chemotherapy regimen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. |
Screening is looking for signs of disease, such as breast cancer, before a person has symptoms. The goal of screening tests is to find
cancer at an early stage when it can be treated and may be cured. Sometimes a screening test finds cancer that is very small or very slow growing. These cancers are unlikely to cause death or illness during the person's lifetime. Scientists are trying to better understand which
people are more likely to get certain types of cancer. For example, they look at the person's age, their family history, and certain exposures during their lifetime. This
information helps doctors recommend who should be screened for cancer, which
screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily
think you have cancer if he or she suggests a screening test. Screening
tests are done when you have no cancer symptoms. Women who have a strong family history or a personal history of cancer or other risk factors may also be offered genetic testing. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests, rather than screening tests.
See the following PDQ summary for more information about cancer screening: The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called
lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that
can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called
ducts.EnlargeAnatomy of the female breast. The nipple and areola are shown on the outside of the breast. The lymph nodes, lobes, lobules, ducts, and other parts of the inside of the breast are also shown. Each breast also has blood
vessels and lymph
vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph
vessels carry lymph between lymph
nodes. Lymph nodes are small, bean-shaped structures that filter lymph and store white blood cells that help fight
infection and disease. Groups of lymph nodes are found near the breast in the
axilla (under the arm), above the
collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: Women in the United States get breast cancer more than any other
type of cancer except for skin cancer. Breast cancer is more likely to occur as a woman ages. It occurs more often in White women than in Black women, but Black women die from breast cancer more often than White women. Breast cancer rarely occurs in men. Because men with breast cancer usually have a lump that can be felt, screening tests are not likely to be helpful. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person’s chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. A mammogram is a picture of the inside of the breast. Mammography may find tumors
that are too small to feel. It may also find ductal carcinoma in situ (DCIS). In DCIS, abnormal cells line the breast duct, and in some women may become invasive cancer. There are three types of mammograms: DBT was approved by the U.S. Food and Drug Administration (FDA) in 2018 and is now used in 3 out of 4 facilities. One study found that DBT reduced false-positive test results (one that shows there is cancer when there really isn’t) and was likely to reduce breast cancer deaths. More studies are being done to provide information on digital mammography compared to DBT. Mammography is less likely to find breast tumors in women with dense breast tissue. Because both tumors and dense breast tissue appear white on a mammogram, it can be harder to find a tumor when there is dense breast tissue. Younger women are more likely to have dense breast tissue.EnlargeMammography. The breast is pressed between two plates. X-rays are used to take pictures of breast tissue. Many factors affect whether mammography is able to detect (find) breast cancer: Women aged 50 to 69 years who have screening mammograms have a lower chance of dying from breast cancer than women who do not have screening mammograms. Fewer women are dying of breast cancer in the United States, but it is not known whether the lower risk of dying is because the cancer was found early by screening or whether the treatments were better. MRI is a procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). MRI does not use any x-rays and the woman is not exposed to radiation. EnlargeAn MRI of the breast is a procedure that uses radio waves, a strong magnet, and a computer to create detailed pictures of the inside of the breast. A contrast dye may be injected into a vein (not shown) to make the breast tissues easier to see on the MRI pictures. An MRI may be used with other breast imaging tests to detect breast cancer or other abnormal changes in the breast. It may also be used to screen for breast cancer in some people who have a high risk of the disease. Note: The inset shows an MRI image of the insides of both breasts. Credit for inset: The Cancer Imaging Archive (TCIA). MRI may be used as a screening test for women who have a high risk of breast cancer. Factors that put women at high risk include the following: An MRI is more likely than mammography to find a breast mass that is not cancer. Women with dense breasts who have supplemental screening (for example, an MRI) show higher rates of breast cancer detection, but there is limited evidence about whether this leads to better health outcomes. Women with risk factors for breast cancer, such as certain changes in the BRCA1 or BRCA2 gene or certain genetic syndromes may be screened at a younger age and more often. Women who have had radiation treatment to the chest, especially at a young age, may start routine breast cancer screening at an earlier age. The benefits and risks of mammograms and MRIs for these women have not been studied. Breast cancer screening has not been shown to benefit the following women: Studies have been done to find out if the following breast cancer screening tests are useful in finding breast cancer or helping women with breast cancer live longer. A clinical breast exam is an exam of the breast by a doctor or other health professional. He or she will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. It is not known if having clinical breast exams decreases the chance of dying from breast cancer. Breast self-exams may be done by women or men to check their breasts for lumps or other changes. If you feel any lumps or notice any other changes in your breasts, talk to your doctor. Doing regular breast self-exams has not been shown to decrease the chance of dying from breast cancer. Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. Tumors can cause temperature changes that may show up on the thermogram. There have been no randomized clinical trials of thermography to find out how well it detects breast cancer or the harms of the procedure. Breast tissue sampling is taking cells from breast tissue to check under a microscope.
Breast tissue sampling as a screening test has not been shown to decrease the risk of dying from breast cancer. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Not all breast cancers will cause death or illness in a woman's lifetime, so they may not need to be found or treated. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have harms. Before having any screening test, you may
want to discuss the test with your doctor. It is important to know the harms of
the test and whether it has been proven to reduce the risk of dying from
cancer. Screening test results may appear to be abnormal even though no
cancer is present. A false-positive test result (one that shows there is cancer when there really isn’t) is usually followed by more tests (such as
biopsy), which also have risks. When a breast biopsy result is abnormal, getting a second opinion from a different pathologist may confirm a correct breast cancer diagnosis. Most abnormal test results turn out not to be
cancer. False-positive results are more common in the following: False-positive results are more likely the first time screening mammography is done than with later screenings. For every ten women who have a single mammogram, one will have a false-positive result. The chance of having a false-positive result goes up the more mammograms a woman has. Comparing a current mammogram with a past mammogram lowers the risk of a false-positive result. The skill of the radiologist also can affect the chance of
a false-positive result. If a mammogram is abnormal, more tests may be done to diagnose cancer. Women can become anxious during the diagnostic testing. Even if it is a false-positive test and cancer is not diagnosed, the result can lead to anxiety anywhere from a few days to years later. Several studies show that women who feel anxiety after false-positive test results are more likely to schedule regular breast screening exams in the future. Screening test results may appear to be normal even though breast cancer is
present. This is called a false-negative test result. A woman who has a false-negative test result may delay seeking medical care even if she has symptoms. About one in 5 cancers are missed by mammography. The chance of a false-negative test result is more common in women who: Some breast cancers found only by screening mammography may never cause health problems or become life-threatening. Finding these cancers is called overdiagnosis. When these cancers are found, having treatment may cause serious side effects and may not lead to a longer, healthier life. Being exposed to high radiation doses is a risk factor for breast
cancer. The radiation dose with a mammogram is very low. Women who start getting mammograms after age 50 have very little risk that the overall exposure to radiation from mammograms throughout their lives will cause harm. Women with large breasts or with breast implants may be exposed to slightly higher radiation doses during screening mammography. During a mammogram, the breast is placed between two plates that are pressed together. Pressing the breast helps to get a better x-ray of the breast. Some women have pain or discomfort during a mammogram. The amount of pain may also depend on the following: Talk to your doctor or other care provider about your risk of breast cancer, whether a screening test is right for you, and the benefits and harms of the screening test. You should take part in the decision about whether you want to have a screening test, based on what is best for you. (See the PDQ summary on Cancer Screening Overview for more information.) Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about breast cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Breast Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/breast/patient/breast-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389160] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Breast Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Breast Cancer ? | Key Points
- Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Breast cancer is the second leading cause of death from cancer in American women. - Different factors increase or decrease the risk of breast cancer.
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also contains blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels lead to organs called lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter substances in lymph and help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: - Breast Cancer Prevention - Breast Cancer Treatment - Genetics of Breast and Gynecologic Cancers
Breast cancer is the second leading cause of death from cancer in American women.
Women in the United States get breast cancer more than any other type of cancer except for skin cancer. Breast cancer is second only to lung cancer as a cause of cancer death in women. Breast cancer occurs more often in white women than in black women. However, black women are more likely than white women to die from the disease. Breast cancer occurs in men also, but the number of cases is small. |
Screening is looking for signs of disease, such as breast cancer, before a person has symptoms. The goal of screening tests is to find
cancer at an early stage when it can be treated and may be cured. Sometimes a screening test finds cancer that is very small or very slow growing. These cancers are unlikely to cause death or illness during the person's lifetime. Scientists are trying to better understand which
people are more likely to get certain types of cancer. For example, they look at the person's age, their family history, and certain exposures during their lifetime. This
information helps doctors recommend who should be screened for cancer, which
screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily
think you have cancer if he or she suggests a screening test. Screening
tests are done when you have no cancer symptoms. Women who have a strong family history or a personal history of cancer or other risk factors may also be offered genetic testing. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests, rather than screening tests.
See the following PDQ summary for more information about cancer screening: The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called
lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that
can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called
ducts.EnlargeAnatomy of the female breast. The nipple and areola are shown on the outside of the breast. The lymph nodes, lobes, lobules, ducts, and other parts of the inside of the breast are also shown. Each breast also has blood
vessels and lymph
vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph
vessels carry lymph between lymph
nodes. Lymph nodes are small, bean-shaped structures that filter lymph and store white blood cells that help fight
infection and disease. Groups of lymph nodes are found near the breast in the
axilla (under the arm), above the
collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: Women in the United States get breast cancer more than any other
type of cancer except for skin cancer. Breast cancer is more likely to occur as a woman ages. It occurs more often in White women than in Black women, but Black women die from breast cancer more often than White women. Breast cancer rarely occurs in men. Because men with breast cancer usually have a lump that can be felt, screening tests are not likely to be helpful. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person’s chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. A mammogram is a picture of the inside of the breast. Mammography may find tumors
that are too small to feel. It may also find ductal carcinoma in situ (DCIS). In DCIS, abnormal cells line the breast duct, and in some women may become invasive cancer. There are three types of mammograms: DBT was approved by the U.S. Food and Drug Administration (FDA) in 2018 and is now used in 3 out of 4 facilities. One study found that DBT reduced false-positive test results (one that shows there is cancer when there really isn’t) and was likely to reduce breast cancer deaths. More studies are being done to provide information on digital mammography compared to DBT. Mammography is less likely to find breast tumors in women with dense breast tissue. Because both tumors and dense breast tissue appear white on a mammogram, it can be harder to find a tumor when there is dense breast tissue. Younger women are more likely to have dense breast tissue.EnlargeMammography. The breast is pressed between two plates. X-rays are used to take pictures of breast tissue. Many factors affect whether mammography is able to detect (find) breast cancer: Women aged 50 to 69 years who have screening mammograms have a lower chance of dying from breast cancer than women who do not have screening mammograms. Fewer women are dying of breast cancer in the United States, but it is not known whether the lower risk of dying is because the cancer was found early by screening or whether the treatments were better. MRI is a procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). MRI does not use any x-rays and the woman is not exposed to radiation. EnlargeAn MRI of the breast is a procedure that uses radio waves, a strong magnet, and a computer to create detailed pictures of the inside of the breast. A contrast dye may be injected into a vein (not shown) to make the breast tissues easier to see on the MRI pictures. An MRI may be used with other breast imaging tests to detect breast cancer or other abnormal changes in the breast. It may also be used to screen for breast cancer in some people who have a high risk of the disease. Note: The inset shows an MRI image of the insides of both breasts. Credit for inset: The Cancer Imaging Archive (TCIA). MRI may be used as a screening test for women who have a high risk of breast cancer. Factors that put women at high risk include the following: An MRI is more likely than mammography to find a breast mass that is not cancer. Women with dense breasts who have supplemental screening (for example, an MRI) show higher rates of breast cancer detection, but there is limited evidence about whether this leads to better health outcomes. Women with risk factors for breast cancer, such as certain changes in the BRCA1 or BRCA2 gene or certain genetic syndromes may be screened at a younger age and more often. Women who have had radiation treatment to the chest, especially at a young age, may start routine breast cancer screening at an earlier age. The benefits and risks of mammograms and MRIs for these women have not been studied. Breast cancer screening has not been shown to benefit the following women: Studies have been done to find out if the following breast cancer screening tests are useful in finding breast cancer or helping women with breast cancer live longer. A clinical breast exam is an exam of the breast by a doctor or other health professional. He or she will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. It is not known if having clinical breast exams decreases the chance of dying from breast cancer. Breast self-exams may be done by women or men to check their breasts for lumps or other changes. If you feel any lumps or notice any other changes in your breasts, talk to your doctor. Doing regular breast self-exams has not been shown to decrease the chance of dying from breast cancer. Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. Tumors can cause temperature changes that may show up on the thermogram. There have been no randomized clinical trials of thermography to find out how well it detects breast cancer or the harms of the procedure. Breast tissue sampling is taking cells from breast tissue to check under a microscope.
Breast tissue sampling as a screening test has not been shown to decrease the risk of dying from breast cancer. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Not all breast cancers will cause death or illness in a woman's lifetime, so they may not need to be found or treated. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have harms. Before having any screening test, you may
want to discuss the test with your doctor. It is important to know the harms of
the test and whether it has been proven to reduce the risk of dying from
cancer. Screening test results may appear to be abnormal even though no
cancer is present. A false-positive test result (one that shows there is cancer when there really isn’t) is usually followed by more tests (such as
biopsy), which also have risks. When a breast biopsy result is abnormal, getting a second opinion from a different pathologist may confirm a correct breast cancer diagnosis. Most abnormal test results turn out not to be
cancer. False-positive results are more common in the following: False-positive results are more likely the first time screening mammography is done than with later screenings. For every ten women who have a single mammogram, one will have a false-positive result. The chance of having a false-positive result goes up the more mammograms a woman has. Comparing a current mammogram with a past mammogram lowers the risk of a false-positive result. The skill of the radiologist also can affect the chance of
a false-positive result. If a mammogram is abnormal, more tests may be done to diagnose cancer. Women can become anxious during the diagnostic testing. Even if it is a false-positive test and cancer is not diagnosed, the result can lead to anxiety anywhere from a few days to years later. Several studies show that women who feel anxiety after false-positive test results are more likely to schedule regular breast screening exams in the future. Screening test results may appear to be normal even though breast cancer is
present. This is called a false-negative test result. A woman who has a false-negative test result may delay seeking medical care even if she has symptoms. About one in 5 cancers are missed by mammography. The chance of a false-negative test result is more common in women who: Some breast cancers found only by screening mammography may never cause health problems or become life-threatening. Finding these cancers is called overdiagnosis. When these cancers are found, having treatment may cause serious side effects and may not lead to a longer, healthier life. Being exposed to high radiation doses is a risk factor for breast
cancer. The radiation dose with a mammogram is very low. Women who start getting mammograms after age 50 have very little risk that the overall exposure to radiation from mammograms throughout their lives will cause harm. Women with large breasts or with breast implants may be exposed to slightly higher radiation doses during screening mammography. During a mammogram, the breast is placed between two plates that are pressed together. Pressing the breast helps to get a better x-ray of the breast. Some women have pain or discomfort during a mammogram. The amount of pain may also depend on the following: Talk to your doctor or other care provider about your risk of breast cancer, whether a screening test is right for you, and the benefits and harms of the screening test. You should take part in the decision about whether you want to have a screening test, based on what is best for you. (See the PDQ summary on Cancer Screening Overview for more information.) Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about breast cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Breast Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/breast/patient/breast-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389160] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Breast Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Breast Cancer? ? | Different factors increase or decrease the risk of breast cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention. |
Screening is looking for signs of disease, such as breast cancer, before a person has symptoms. The goal of screening tests is to find
cancer at an early stage when it can be treated and may be cured. Sometimes a screening test finds cancer that is very small or very slow growing. These cancers are unlikely to cause death or illness during the person's lifetime. Scientists are trying to better understand which
people are more likely to get certain types of cancer. For example, they look at the person's age, their family history, and certain exposures during their lifetime. This
information helps doctors recommend who should be screened for cancer, which
screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily
think you have cancer if he or she suggests a screening test. Screening
tests are done when you have no cancer symptoms. Women who have a strong family history or a personal history of cancer or other risk factors may also be offered genetic testing. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests, rather than screening tests.
See the following PDQ summary for more information about cancer screening: The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called
lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that
can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called
ducts.EnlargeAnatomy of the female breast. The nipple and areola are shown on the outside of the breast. The lymph nodes, lobes, lobules, ducts, and other parts of the inside of the breast are also shown. Each breast also has blood
vessels and lymph
vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph
vessels carry lymph between lymph
nodes. Lymph nodes are small, bean-shaped structures that filter lymph and store white blood cells that help fight
infection and disease. Groups of lymph nodes are found near the breast in the
axilla (under the arm), above the
collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: Women in the United States get breast cancer more than any other
type of cancer except for skin cancer. Breast cancer is more likely to occur as a woman ages. It occurs more often in White women than in Black women, but Black women die from breast cancer more often than White women. Breast cancer rarely occurs in men. Because men with breast cancer usually have a lump that can be felt, screening tests are not likely to be helpful. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person’s chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. A mammogram is a picture of the inside of the breast. Mammography may find tumors
that are too small to feel. It may also find ductal carcinoma in situ (DCIS). In DCIS, abnormal cells line the breast duct, and in some women may become invasive cancer. There are three types of mammograms: DBT was approved by the U.S. Food and Drug Administration (FDA) in 2018 and is now used in 3 out of 4 facilities. One study found that DBT reduced false-positive test results (one that shows there is cancer when there really isn’t) and was likely to reduce breast cancer deaths. More studies are being done to provide information on digital mammography compared to DBT. Mammography is less likely to find breast tumors in women with dense breast tissue. Because both tumors and dense breast tissue appear white on a mammogram, it can be harder to find a tumor when there is dense breast tissue. Younger women are more likely to have dense breast tissue.EnlargeMammography. The breast is pressed between two plates. X-rays are used to take pictures of breast tissue. Many factors affect whether mammography is able to detect (find) breast cancer: Women aged 50 to 69 years who have screening mammograms have a lower chance of dying from breast cancer than women who do not have screening mammograms. Fewer women are dying of breast cancer in the United States, but it is not known whether the lower risk of dying is because the cancer was found early by screening or whether the treatments were better. MRI is a procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). MRI does not use any x-rays and the woman is not exposed to radiation. EnlargeAn MRI of the breast is a procedure that uses radio waves, a strong magnet, and a computer to create detailed pictures of the inside of the breast. A contrast dye may be injected into a vein (not shown) to make the breast tissues easier to see on the MRI pictures. An MRI may be used with other breast imaging tests to detect breast cancer or other abnormal changes in the breast. It may also be used to screen for breast cancer in some people who have a high risk of the disease. Note: The inset shows an MRI image of the insides of both breasts. Credit for inset: The Cancer Imaging Archive (TCIA). MRI may be used as a screening test for women who have a high risk of breast cancer. Factors that put women at high risk include the following: An MRI is more likely than mammography to find a breast mass that is not cancer. Women with dense breasts who have supplemental screening (for example, an MRI) show higher rates of breast cancer detection, but there is limited evidence about whether this leads to better health outcomes. Women with risk factors for breast cancer, such as certain changes in the BRCA1 or BRCA2 gene or certain genetic syndromes may be screened at a younger age and more often. Women who have had radiation treatment to the chest, especially at a young age, may start routine breast cancer screening at an earlier age. The benefits and risks of mammograms and MRIs for these women have not been studied. Breast cancer screening has not been shown to benefit the following women: Studies have been done to find out if the following breast cancer screening tests are useful in finding breast cancer or helping women with breast cancer live longer. A clinical breast exam is an exam of the breast by a doctor or other health professional. He or she will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. It is not known if having clinical breast exams decreases the chance of dying from breast cancer. Breast self-exams may be done by women or men to check their breasts for lumps or other changes. If you feel any lumps or notice any other changes in your breasts, talk to your doctor. Doing regular breast self-exams has not been shown to decrease the chance of dying from breast cancer. Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. Tumors can cause temperature changes that may show up on the thermogram. There have been no randomized clinical trials of thermography to find out how well it detects breast cancer or the harms of the procedure. Breast tissue sampling is taking cells from breast tissue to check under a microscope.
Breast tissue sampling as a screening test has not been shown to decrease the risk of dying from breast cancer. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Not all breast cancers will cause death or illness in a woman's lifetime, so they may not need to be found or treated. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have harms. Before having any screening test, you may
want to discuss the test with your doctor. It is important to know the harms of
the test and whether it has been proven to reduce the risk of dying from
cancer. Screening test results may appear to be abnormal even though no
cancer is present. A false-positive test result (one that shows there is cancer when there really isn’t) is usually followed by more tests (such as
biopsy), which also have risks. When a breast biopsy result is abnormal, getting a second opinion from a different pathologist may confirm a correct breast cancer diagnosis. Most abnormal test results turn out not to be
cancer. False-positive results are more common in the following: False-positive results are more likely the first time screening mammography is done than with later screenings. For every ten women who have a single mammogram, one will have a false-positive result. The chance of having a false-positive result goes up the more mammograms a woman has. Comparing a current mammogram with a past mammogram lowers the risk of a false-positive result. The skill of the radiologist also can affect the chance of
a false-positive result. If a mammogram is abnormal, more tests may be done to diagnose cancer. Women can become anxious during the diagnostic testing. Even if it is a false-positive test and cancer is not diagnosed, the result can lead to anxiety anywhere from a few days to years later. Several studies show that women who feel anxiety after false-positive test results are more likely to schedule regular breast screening exams in the future. Screening test results may appear to be normal even though breast cancer is
present. This is called a false-negative test result. A woman who has a false-negative test result may delay seeking medical care even if she has symptoms. About one in 5 cancers are missed by mammography. The chance of a false-negative test result is more common in women who: Some breast cancers found only by screening mammography may never cause health problems or become life-threatening. Finding these cancers is called overdiagnosis. When these cancers are found, having treatment may cause serious side effects and may not lead to a longer, healthier life. Being exposed to high radiation doses is a risk factor for breast
cancer. The radiation dose with a mammogram is very low. Women who start getting mammograms after age 50 have very little risk that the overall exposure to radiation from mammograms throughout their lives will cause harm. Women with large breasts or with breast implants may be exposed to slightly higher radiation doses during screening mammography. During a mammogram, the breast is placed between two plates that are pressed together. Pressing the breast helps to get a better x-ray of the breast. Some women have pain or discomfort during a mammogram. The amount of pain may also depend on the following: Talk to your doctor or other care provider about your risk of breast cancer, whether a screening test is right for you, and the benefits and harms of the screening test. You should take part in the decision about whether you want to have a screening test, based on what is best for you. (See the PDQ summary on Cancer Screening Overview for more information.) Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about breast cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Breast Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/breast/patient/breast-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389160] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Breast Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Breast Cancer ? | Other screening tests are being studied in clinical trials.
Thermography Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. A computer makes a map of the breast showing the changes in temperature. Tumors can cause temperature changes that may show up on the thermogram. There have been no clinical trials of thermography to find out how well it detects breast cancer or if having the procedure decreases the risk of dying from breast cancer. Tissue sampling Breast tissue sampling is taking cells from breast tissue to check under a microscope. Abnormal cells in breast fluid have been linked to an increased risk of breast cancer in some studies. Scientists are studying whether breast tissue sampling can be used to find breast cancer at an early stage or predict the risk of developing breast cancer. Three ways of taking tissue samples are being studied: - Fine-needle aspiration: A thin needle is inserted into the breast tissue around the areola (darkened area around the nipple) to take out a sample of cells and fluid. - Nipple aspiration: The use of gentle suction to collect fluid through the nipple. This is done with a device similar to the breast pumps used by women who are breast-feeding. - Ductal lavage: A hair-size catheter (tube) is inserted into the nipple and a small amount of salt water is released into the duct. The water picks up breast cells and is removed. Screening clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Adult Acute Lymphoblastic Leukemia ? | Key Points
- Adult acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Previous chemotherapy and exposure to radiation may increase the risk of developing ALL. - Signs and symptoms of adult ALL include fever, feeling tired, and easy bruising or bleeding. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult ALL. - Certain factors affect prognosis (chance of recovery) and treatment options.
Adult acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated.
Leukemia may affect red blood cells, white blood cells, and platelets.
Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - Platelets that form blood clots to stop bleeding. - Granulocytes (white blood cells) that fight infection and disease. A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make the antibodies that help fight infection. - Natural killer cells that attack cancer cells and viruses. In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal cord). This summary is about adult acute lymphoblastic leukemia. See the following PDQ summaries for information about other types of leukemia: - Childhood Acute Lymphoblastic Leukemia Treatment. - Adult Acute Myeloid Leukemia Treatment. - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment. - Chronic Lymphocytic Leukemia Treatment. - Chronic Myelogenous Leukemia Treatment. - Hairy Cell Leukemia Treatment. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Adult Acute Lymphoblastic Leukemia ? | Signs and symptoms of adult ALL include fever, feeling tired, and easy bruising or bleeding. The early signs and symptoms of ALL may be like the flu or other common diseases. Check with your doctor if you have any of the following: - Weakness or feeling tired. - Fever or night sweats. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin, caused by bleeding). - Shortness of breath. - Weight loss or loss of appetite. - Pain in the bones or stomach. - Pain or feeling of fullness below the ribs. - Painless lumps in the neck, underarm, stomach, or groin. - Having many infections. These and other signs and symptoms may be caused by adult acute lymphoblastic leukemia or by other conditions. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Adult Acute Lymphoblastic Leukemia ? | Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult ALL. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as infection or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. The following tests may be done on the samples of blood or bone marrow tissue that are removed: - Cytogenetic analysis: A laboratory test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if there are certain changes in the chromosomes of lymphocytes. For example, in Philadelphia chromosome positive ALL, part of one chromosome switches places with part of another chromosome. This is called the Philadelphia chromosome. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose the subtype of ALL by comparing the cancer cells to normal cells of the immune system. For example, a cytochemistry study may test the cells in a sample of tissue using chemicals (dyes) to look for certain changes in the sample. A chemical may cause a color change in one type of leukemia cell but not in another type of leukemia cell. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Adult Acute Lymphoblastic Leukemia ? | Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The age of the patient. - Whether the cancer has spread to the brain or spinal cord. - Whether there are certain changes in the genes, including the Philadelphia chromosome. - Whether the cancer has been treated before or has recurred (come back). |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Adult Acute Lymphoblastic Leukemia? ? | Previous chemotherapy and exposure to radiation may increase the risk of developing ALL. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: - Being male. - Being white. - Being older than 70. - Past treatment with chemotherapy or radiation therapy. - Being exposed to high levels of radiation in the environment (such as nuclear radiation). - Having certain genetic disorders, such as Down syndrome. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Adult Acute Lymphoblastic Leukemia ? | Key Points
- Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body. - There is no standard staging system for adult ALL.
Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body.
The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to determine if the leukemia has spread: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
There is no standard staging system for adult ALL.
The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. - The complete blood count is abnormal. - More than 5% of the cells in the bone marrow are blasts (leukemia cells). - There are signs and symptoms of leukemia. Adult ALL in remission The ALL has been treated. - The complete blood count is normal. - 5% or fewer of the cells in the bone marrow are blasts (leukemia cells). - There are no signs or symptoms of leukemia other than in the bone marrow. |
Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic
leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal
cord), lymph nodes, spleen, liver, testicles, and other organs. This summary is about adult acute lymphoblastic leukemia.
See the following PDQ summaries for information about other types of leukemia: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: The early signs and symptoms of ALL may be like the flu or other common diseases.
Check with your doctor if you have any of the following: These and other signs and symptoms may be caused by adult acute lymphoblastic
leukemia or by other conditions. The following tests and procedures may be used:
The following tests may be done on the samples of blood or bone marrow tissue that are removed: The prognosis and treatment options depend on the following: The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to find out if the leukemia has spread: The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. Adult ALL in remission The ALL has been treated. Recurrent adult ALL Recurrent adult ALL is cancer that has recurred (come back) after
going into remission. The ALL may come back in the blood, bone
marrow, or other parts of the body. Different types of treatment are available for patients with adult
acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are
being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL is done in phases: Treatment called central nervous system (CNS) prophylaxis therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the leukemia cells are able to hide in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. These treatments are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called CNS prophylaxis. EnlargeIntrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Total-body irradiation may be used to send radiation toward the whole body when preparing for a stem cell transplant. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy is given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy or total-body radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. This summary section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the
NCI website. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of adult acute lymphoblastic leukemia (ALL) during the post-remission phase includes the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Standard treatment of recurrent adult acute lymphoblastic leukemia (ALL) may include the following: Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about adult acute lymphoblastic leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult acute lymphoblastic leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389283] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Adult Acute Lymphoblastic Leukemia ? | Key Points
- There are different types of treatment for patients with adult ALL. - The treatment of adult ALL usually has two phases. - Four types of standard treatment are used: - Chemotherapy - Radiation therapy - Chemotherapy with stem cell transplant - Targeted therapy - New types of treatment are being tested in clinical trials. - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Patients with ALL may have late effects after treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with adult ALL.
Different types of treatment are available for patients with adult acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
The treatment of adult ALL usually has two phases.
The treatment of adult ALL is done in phases: - Remission induction therapy: This is the first phase of treatment. The goal is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. - Post-remission therapy: This is the second phase of treatment. It begins once the leukemia is in remission. The goal of post-remission therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy. Treatment called central nervous system (CNS) sanctuary therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the cells are able to "find sanctuary" (hide) in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. They are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). CNS sanctuary therapy is also called CNS prophylaxis.
Four types of standard treatment are used:
Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy with stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy drugs called tyrosine kinase inhibitors are used to treat some types of adult ALL. These drugs block the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (blasts) than the body needs. Three of the drugs used are imatinib mesylate (Gleevec), dasatinib, and nilotinib. See Drugs Approved for Acute Lymphoblastic Leukemia for more information.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Patients with ALL may have late effects after treatment.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Adult Acute Lymphoblastic Leukemia
Untreated Adult Acute Lymphoblastic Leukemia
Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: - Combination chemotherapy. - Tyrosine kinase inhibitor therapy with imatinib mesylate, in certain patients. Some of these patients will also have combination chemotherapy. - Supportive care including antibiotics and red blood cell and platelet transfusions. - CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated adult acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Adult Acute Lymphoblastic Leukemia in Remission
Standard treatment of adult ALL during the post-remission phase includes the following: - Chemotherapy. - Tyrosine kinase inhibitor therapy. - Chemotherapy with stem cell transplant. - CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult acute lymphoblastic leukemia in remission. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Recurrent Adult Acute Lymphoblastic Leukemia
Standard treatment of recurrent adult ALL may include the following: - Combination chemotherapy followed by stem cell transplant. - Low-dose radiation therapy as palliative care to relieve symptoms and improve the quality of life. - Tyrosine kinase inhibitor therapy with dasatinib for certain patients. Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: - A clinical trial of stem cell transplant using the patient's stem cells. - A clinical trial of biologic therapy. - A clinical trial of new anticancer drugs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Retinoblastoma ? | Key Points
- Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. - Retinoblastoma occurs in heritable and nonheritable forms. - Treatment for both forms of retinoblastoma should include genetic counseling. - Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma. - A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers. - Signs and symptoms of retinoblastoma include "white pupil" and eye pain or redness. - Tests that examine the retina are used to detect (find) and diagnose retinoblastoma. - Certain factors affect prognosis (chance of recovery) and treatment options.
Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.
The retina is the nerve tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. The cancer may be in one eye (unilateral) or in both eyes (bilateral). Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body. Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor.
Treatment for both forms of retinoblastoma should include genetic counseling.
Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) to discuss genetic testing to check for a mutation (change) in the RB1 gene. Genetic counseling also includes a discussion of the risk of retinoblastoma for the child and the child's brothers or sisters. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Is Retinoblastoma inherited ? | Retinoblastoma occurs in heritable and nonheritable forms.
A child is thought to have the heritable form of retinoblastoma when one of the following is true: - There is a family history of retinoblastoma. - There is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child or it may occur in the egg or sperm before conception or soon after conception. - There is more than one tumor in the eye or there is a tumor in both eyes. - There is a tumor in one eye and the child is younger than 1 year. After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Nonheritable retinoblastoma is retinoblastoma that is not the heritable form. Most cases of retinoblastoma are the nonheritable form.
Treatment for both forms of retinoblastoma should include genetic counseling.
Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) to discuss genetic testing to check for a mutation (change) in the RB1 gene. Genetic counseling also includes a discussion of the risk of retinoblastoma for the child and the child's brothers or sisters.
Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma.
A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Brothers or sisters of a child with retinoblastoma should have regular eye exams by an ophthalmologist until age 3 to 5 years, unless it is known that the brother or sister does not have the RB1 gene change.
A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers.
A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Retinoblastoma? ? | A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Retinoblastoma ? | These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: - Pupil of the eye appears white instead of red when light shines into it. This may be seen in flash photographs of the child. - Eyes appear to be looking in different directions (lazy eye). - Pain or redness in the eye. - Infection around the eye. - Eyeball is larger than normal. - Colored part of the eye and pupil look cloudy. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Retinoblastoma ? | The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. The doctor will ask if there is a family history of retinoblastoma. - Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (opened wider) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is examined with a light. Depending on the age of the child, this exam may be done under anesthesia. There are several types of eye exams that are done with the pupil dilated: - Ophthalmoscopy : An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light. - Slit-lamp biomicroscopy : An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope. - Fluorescein angiography : A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye called fluorescein is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any blood vessels that are blocked or leaking. - RB1 gene test: A laboratory test in which a sample of blood or tissue is tested for a change in the RB1 gene. - Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor. The picture can be printed to be looked at later. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the eye. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the eye, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known if the retinoblastoma is the heritable form. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Retinoblastoma ? | The prognosis (chance of recovery) and treatment options depend on the following: - Whether the cancer is in one or both eyes. - The size and number of tumors. - Whether the tumor has spread to the area around the eye, to the brain, or to other parts of the body. - Whether there are symptoms at the time of diagnosis, for trilateral retinoblastoma. - The age of the child. - How likely it is that vision can be saved in one or both eyes. - Whether a second type of cancer has formed. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Retinoblastoma ? | Key Points
- After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body. - The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma. - Stage 0 - Stage I - Stage II - Stage III - Stage IV - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye). - Intraocular retinoblastoma - Extraocular retinoblastoma (metastatic)
After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body.
The process used to find out if cancer has spread within the eye or to other parts of the body is called staging. The information gathered from the staging process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.) The following tests and procedures may be used in the staging process: - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner that also takes a picture of the body. Areas of bone with cancer show up brighter in the picture because they take up more radioactive material than normal bone cells do. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow under a microscope to look for signs of cancer. A bone marrow aspiration and biopsy is done if the doctor thinks the cancer has spread outside of the eye. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap.
The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma.
There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread. Stage 0 The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery. Stage I The tumor is in the eye only. The eye has been removed and no cancer cells remain. Stage II The tumor is in the eye only. The eye has been removed and there are cancer cells left that can be seen only with a microscope. Stage III Stage III is divided into stages IIIa and IIIb: - In stage IIIa, cancer has spread from the eye to tissues around the eye socket. - In stage IIIb, cancer has spread from the eye to lymph nodes near the ear or in the neck. Stage IV Stage IV is divided into stages IVa and IVb: - In stage IVa, cancer has spread to the blood but not to the brain or spinal cord. One or more tumors may have spread to other parts of the body such as the bone or liver. - In stage IVb, cancer has spread to the brain or spinal cord. It also may have spread to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer.
Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye).
Intraocular retinoblastoma In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. Extraocular retinoblastoma (metastatic) In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma) or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bones, bone marrow, or lymph nodes. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Retinoblastoma ? | Key Points
- There are different types of treatment for patients with retinoblastoma. - Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for retinoblastoma may cause side effects. - Six types of standard treatment are used: - Cryotherapy - Thermotherapy - Chemotherapy - Radiation therapy - High-dose chemotherapy with stem cell rescue - Surgery (enucleation) - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with retinoblastoma.
Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with eye cancer and who specialize in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following specialists: - Pediatric surgeon. - Radiation oncologist. - Pediatrician. - Pediatric nurse specialist. - Rehabilitation specialist. - Social worker. - Geneticist or genetic counselor.
Treatment for retinoblastoma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following: - Physical problems such as seeing or hearing problems or, if the eye is removed, a change in the shape and size of the bone around the eye. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer), such as lung and bladder cancers, osteosarcoma, soft tissue sarcoma, or melanoma. The following risk factors may increase the risk of having another cancer: - Having the heritable form of retinoblastoma. - Past treatment with radiation therapy, especially before age 1 year. - Having already had a previous second cancer. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.
Six types of standard treatment are used:
Cryotherapy Cryotherapy is a treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryosurgery. Thermotherapy Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: - Systemic chemotherapy: When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body. Systemic chemotherapy is given to shrink the tumor (chemoreduction) and avoid surgery to remove the eye. After chemoreduction, other treatments may include radiation therapy, cryotherapy, laser therapy, or regional chemotherapy. Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to patients with retinoblastoma that occurs outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. - Regional chemotherapy: When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ (such as the eye), or a body cavity, the drugs mainly affect cancer cells in those areas. Several types of regional chemotherapy are used to treat retinoblastoma. - Ophthalmic artery infusion chemotherapy: Ophthalmic artery infusion chemotherapy carries anticancer drugs directly to the eye. A catheter is put into an artery that leads to the eye and the anticancer drug is given through the catheter. After the drug is given, a small balloon may be inserted into the artery to block it and keep most of the anticancer drug trapped near the tumor. This type of chemotherapy may be given as the initial treatment when the tumor is in the eye only or when the tumor has not responded to other types of treatment. Ophthalmic artery infusion chemotherapy is given at special retinoblastoma treatment centers. - Intravitreal chemotherapy: Intravitreal chemotherapy is the injection of anticancer drugs directly into the vitreous humor (jelly-like substance) inside in the eye. It is used to treat cancer that has spread to the vitreous humor and has not responded to treatment or has come back after treatment. See Drugs Approved for Retinoblastoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External-beam radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) external radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Proton-beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of internal radiation therapy may include the following: - Plaque radiotherapy: Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation. The way the radiation therapy is given depends on the type and stage of the cancer being treated and how the cancer responded to other treatments. External and internal radiation therapy are used to treat retinoblastoma. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Retinoblastoma for more information. Surgery (enucleation) Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Retinoblastoma
Treatment of Unilateral, Bilateral, and Cavitary Retinoblastoma
If it is likely that the eye can be saved, treatment may include the following: - Systemic chemotherapy or ophthalmic artery infusion chemotherapy, with or without intravitreal chemotherapy, to shrink the tumor. This may be followed by one or more of the following: - Cryotherapy. - Thermotherapy. - Plaque radiotherapy. - External-beam radiation therapy for bilateral intraocular retinoblastoma that does not respond to other treatments. - A clinical trial of ophthalmic artery infusion for unilateral retinoblastoma that has spread to the vitreous humor. If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: - Surgery (enucleation). After surgery, systemic chemotherapy may be given to lower the risk that the cancer will spread to other parts of the body. When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Treatment for cavitary retinoblastoma, a type of intraocular retinoblastoma, may include the following: - Systemic chemotherapy or ophthalmic artery infusion chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with intraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
Treatment of Extraocular Retinoblastoma
Treatment for extraocular retinoblastoma that has spread to the area around the eye may include the following: - Systemic chemotherapy and external-beam radiation therapy. - Systemic chemotherapy followed by surgery (enucleation). External-beam radiation therapy and more chemotherapy may be given after surgery. Treatment for extraocular retinoblastoma that has spread to the brain may include the following: - Systemic or intrathecal chemotherapy. - External-beam radiation therapy to the brain and spinal cord. - Chemotherapy followed by high-dose chemotherapy with stem cell rescue. It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma, treatment may include the following: - Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue. - Systemic chemotherapy followed by surgery and external-beam radiation therapy. For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: - Chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
Treatment of Progressive or Recurrent Retinoblastoma
Treatment of progressive or recurrent intraocular retinoblastoma may include the following: - External-beam radiation therapy or plaque radiotherapy. - Cryotherapy. - Thermotherapy. - Systemic chemotherapy or ophthalmic artery infusion chemotherapy. - Intravitreal chemotherapy. - Surgery (enucleation). Treatment of progressive or recurrent extraocular retinoblastoma may include the following: - Systemic chemotherapy and external-beam radiation therapy for retinoblastoma that comes back after surgery to remove the eye. - Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. |
The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image. EnlargeAnatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye. Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop. A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception. Other factors that suggest the child may have the heritable form of retinoblastoma include the following: After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important. It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following: Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members. Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father. These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: The following tests and procedures may be used: There are several types of eye exams that are done with the pupil dilated: Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the eye or
to other parts of the body is called staging. The information gathered from the staging
process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know
the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease. The following tests and procedures may be used in the staging process: There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
The tumor is in the eye only. The eye has been removed and no cancer cells remain.
The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.
Stage III is divided into stage IIIa and stage IIIb: Stage IV is divided into stage IVa and stage IVb: Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes. Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some
are being tested in clinical trials.
A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a
pediatric oncologist, a doctor who specializes
in treating children with cancer. The pediatric oncologist works with
other health care providers who are experts in treating children
with eye cancer and who specialize
in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following
specialists: Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery. Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Retinoblastoma for more information. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy. See Drugs Approved for Retinoblastoma for more information. Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:
The following risk factors may increase the risk of having a second cancer: It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain. For information about the treatments listed below, see the Treatment Option Overview section. If it is likely that the eye can be saved, treatment may include the following: If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following: Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following: It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following: For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of progressive or recurrent intraocular retinoblastoma may include the following: Treatment of progressive or recurrent extraocular retinoblastoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about the treatment of retinoblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of retinoblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Retinoblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389197] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Retinoblastoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Retinoblastoma ? | New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
What is (are) Lip and Oral Cavity Cancer ? | Key Points
- Lip and oral cavity cancer is a disease in which malignant (cancer) cells form in the lips or mouth. - Tobacco and alcohol use can affect the risk of lip and oral cavity cancer. - Signs of lip and oral cavity cancer include a sore or lump on the lips or in the mouth. - Tests that examine the mouth and throat are used to detect (find), diagnose, and stage lip and oral cavity cancer. - Certain factors affect prognosis (chance of recovery) and treatment options.
Lip and oral cavity cancer is a disease in which malignant (cancer) cells form in the lips or mouth.
The oral cavity includes the following: - The front two thirds of the tongue. - The gingiva (gums). - The buccal mucosa (the lining of the inside of the cheeks). - The floor (bottom) of the mouth under the tongue. - The hard palate (the roof of the mouth). - The retromolar trigone (the small area behind the wisdom teeth). Most lip and oral cavity cancers start in squamous cells, the thin, flat cells that line the lips and oral cavity. These are called squamous cell carcinomas. Cancer cells may spread into deeper tissue as the cancer grows. Squamous cell carcinoma usually develops in areas of leukoplakia (white patches of cells that do not rub off). Lip and oral cavity cancer is a type of head and neck cancer. | |
Who is at risk for Lip and Oral Cavity Cancer? ? | Tobacco and alcohol use can affect the risk of lip and oral cavity cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for lip and oral cavity cancer include the following: - Using tobacco products. - Heavy alcohol use. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Being male. | |
What are the symptoms of Lip and Oral Cavity Cancer ? | Signs of lip and oral cavity cancer include a sore or lump on the lips or in the mouth. These and other signs and symptoms may be caused by lip and oral cavity cancer or by other conditions. Check with your doctor if you have any of the following: - A sore on the lip or in the mouth that does not heal. - A lump or thickening on the lips or gums or in the mouth. - A white or red patch on the gums, tongue, or lining of the mouth. - Bleeding, pain, or numbness in the lip or mouth. - Change in voice. - Loose teeth or dentures that no longer fit well. - Trouble chewing or swallowing or moving the tongue or jaw. - Swelling of jaw. - Sore throat or feeling that something is caught in the throat. Lip and oral cavity cancer may not have any symptoms and is sometimes found during a regular dental exam. | |
How to diagnose Lip and Oral Cavity Cancer ? | Tests that examine the mouth and throat are used to detect (find), diagnose, and stage lip and oral cavity cancer. The following tests and procedures may be used: - Physical exam of the lips and oral cavity: An exam to check the lips and oral cavity for abnormal areas. The medical doctor or dentist will feel the entire inside of the mouth with a gloved finger and examine the oral cavity with a small long-handled mirror and lights. This will include checking the insides of the cheeks and lips; the gums; the roof and floor of the mouth; and the top, bottom, and sides of the tongue. The neck will be felt for swollen lymph nodes. A history of the patients health habits and past illnesses and medical and dental treatments will also be taken. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist. If leukoplakia is found, cells taken from the patches are also checked under the microscope for signs of cancer. - Exfoliative cytology : A procedure to collect cells from the lip or oral cavity. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the lips, tongue, mouth, or throat. The cells are viewed under a microscope to find out if they are abnormal. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and x-rays are taken. This procedure is also called an upper GI series. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. | |
What is the outlook for Lip and Oral Cavity Cancer ? | Certain factors affect prognosis (chance of recovery) and treatment options. Prognosis (chance of recovery) depends on the following: - The stage of the cancer. - Where the tumor is in the lip or oral cavity. - Whether the cancer has spread to blood vessels. For patients who smoke, the chance of recovery is better if they stop smoking before beginning radiation therapy. Treatment options depend on the following: - The stage of the cancer. - The size of the tumor and where it is in the lip or oral cavity. - Whether the patient's appearance and ability to talk and eat can stay the same. - The patient's age and general health. Patients who have had lip and oral cavity cancer have an increased risk of developing a second cancer in the head or neck. Frequent and careful follow-up is important. Clinical trials are studying the use of retinoid drugs to reduce the risk of a second head and neck cancer. Information about ongoing clinical trials is available from the NCI website. | |
What are the stages of Lip and Oral Cavity Cancer ? | Key Points
- After lip and oral cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lip and oral cavity or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for lip and oral cavity cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV
After lip and oral cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lip and oral cavity or to other parts of the body.
The process used to find out if cancer has spread within the lip and oral cavity or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose lip and oral cavity cancer are also used to stage the disease. (See the General Information section.)
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if lip cancer spreads to the lung, the cancer cells in the lung are actually lip cancer cells. The disease is metastatic lip cancer, not lung cancer.
The following stages are used for lip and oral cavity cancer:
Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the lips and oral cavity. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and the tumor is 2 centimeters or smaller. Cancer has not spread to the lymph nodes. Stage II In stage II, the tumor is larger than 2 centimeters but not larger than 4 centimeters, and cancer has not spread to the lymph nodes. Stage III In stage III, the tumor: - may be any size and has spread to one lymph node that is 3 centimeters or smaller, on the same side of the neck as the tumor; or - is larger than 4 centimeters. Stage IV Stage IV is divided into stages IVA, IVB, and IVC. - In stage IVA, the tumor: - has spread through tissue in the lip or oral cavity into nearby tissue and/or bone (jaw, tongue, floor of mouth, maxillary sinus, or skin on the chin or nose); cancer may have spread to one lymph node that is 3 centimeters or smaller, on the same side of the neck as the tumor; or - is any size or has spread through tissue in the lip or oral cavity into nearby tissue and/or bone (jaw, tongue, floor of mouth, maxillary sinus, or skin on the chin or nose), and cancer has spread: - to one lymph node on the same side of the neck as the tumor and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. - In stage IVB, the tumor: - may be any size and has spread to one or more lymph nodes that are larger than 6 centimeters; or - has spread further into the muscles or bones in the oral cavity, or to the base of the skull and/or the carotid artery. Cancer may have spread to one or more lymph nodes anywhere in the neck. - In stage IVC, the tumor has spread beyond the lip or oral cavity to distant parts of the body, such as the lungs. The tumor may be any size and may have spread to the lymph nodes. | |
What are the treatments for Lip and Oral Cavity Cancer ? | Key Points
- There are different types of treatment for patients with lip and oral cavity cancer. - Patients with lip and oral cavity cancer should have their treatment planned by a team of doctors who are expert in treating head and neck cancer. - Two types of standard treatment are used: - Surgery - Radiation therapy - New types of treatment are being tested in clinical trials. - Chemotherapy - Hyperfractionated radiation therapy - Hyperthermia therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with lip and oral cavity cancer.
Different types of treatment are available for patients with lip and oral cavity cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Patients with lip and oral cavity cancer should have their treatment planned by a team of doctors who are expert in treating head and neck cancer.
Treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the lips and oral cavity are important for breathing, eating, and talking, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer the patient to other health professionals with special training in the treatment of patients with head and neck cancer. These include the following: - Head and neck surgeon. - Radiation oncologist. - Dentist. - Speech therapist. - Dietitian. - Psychologist. - Rehabilitation specialist. - Plastic surgeon.
Two types of standard treatment are used:
Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of lip and oral cavity cancer. Surgery may include the following: - Wide local excision: Removal of the cancer and some of the healthy tissue around it. If cancer has spread into bone, surgery may include removal of the involved bone tissue. - Neck dissection: Removal of lymph nodes and other tissues in the neck. This is done when cancer may have spread from the lip and oral cavity. - Plastic surgery: An operation that restores or improves the appearance of parts of the body. Dental implants, a skin graft, or other plastic surgery may be needed to repair parts of the mouth, throat, or neck after removal of large tumors. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat lip and oral cavity cancer. Radiation therapy may work better in patients who have stopped smoking before beginning treatment. It is also important for patients to have a dental exam before radiation therapy begins, so that existing problems can be treated.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hyperfractionated radiation therapy Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. Hyperthermia therapy Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Stage
Stage I Lip and Oral Cavity Cancer
Treatment of stage I lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery (wide local excision). - Internal radiation therapy with or without external radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Surgery (wide local excision). - Internal radiation therapy with or without external radiation therapy. - Radiation therapy to lymph nodes in the neck. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) for tumors smaller than 1 centimeter, with or without internal and/or external radiation therapy. - Surgery (wide local excision with skin graft) or radiation therapy for larger tumors. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision) for tumors smaller than centimeter. - Surgery (wide local excision) or radiation therapy for larger tumors. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, and skin graft). - Radiation therapy with or without surgery. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone.) - Radiation therapy with or without surgery. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or the hard palate (the roof of the mouth), treatment is usually surgery (wide local excision) with or without radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage II Lip and Oral Cavity Cancer
Treatment of stage II lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery (wide local excision). - External radiation therapy and/or internal radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Radiation therapy and/or surgery (wide local excision). - Internal radiation therapy with surgery (neck dissection). Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Radiation therapy for tumors that are 3 centimeters or smaller. - Surgery (wide local excision) and/or radiation therapy for larger tumors. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision). - Radiation therapy. - Surgery (wide local excision) followed by external radiation therapy, with or without internal radiation therapy, for large tumors. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, and a skin graft). - Radiation therapy alone or after surgery. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery (wide local excision, which includes removing part of the jawbone). - Radiation therapy with or without surgery. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or the hard palate (the roof of the mouth), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. - Radiation therapy alone. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage III Lip and Oral Cavity Cancer
Treatment of stage III lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery and external radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - External radiation therapy with or without internal radiation therapy. - Surgery (wide local excision) followed by radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. - Radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, with or without neck dissection). - External radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. Radiation may be given before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery to remove the tumor, lymph nodes, and part of the jawbone, with or without radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Upper gingiva If cancer is in the upper gingiva (gums), treatment may include the following: - Radiation therapy. - Surgery (wide local excision) and radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Hard palate If cancer is in the hard palate (the roof of the mouth), treatment may include the following: - Radiation therapy. - Surgery (wide local excision) with or without radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Lymph nodes For cancer that may have spread to lymph nodes, treatment may include the following: - Radiation therapy and/or surgery (neck dissection). - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage IV Lip and Oral Cavity Cancer
Treatment of stage IV lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery and external radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Surgery to remove the tongue and sometimes the larynx (voice box) with or without radiation therapy. - Radiation therapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) and/or radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery before or after radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery and/or radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery to remove the tumor, lymph nodes, and part of the jawbone, followed by radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or hard palate (the roof of the mouth), treatment may include the following: - Surgery with radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Lymph nodes For cancer that may have spread to lymph nodes, treatment may include the following: - Radiation therapy and/or surgery (neck dissection). - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. | |
what research (or clinical trials) is being done for Lip and Oral Cavity Cancer ? | New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hyperfractionated radiation therapy Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. Hyperthermia therapy Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. | |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Adult Hodgkin Lymphoma ? | Key Points
- Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant. - Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. - Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. - Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options.
Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
Adult Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. Cancer can spread to the liver and lungs. Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma. (See the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.) Hodgkin lymphoma can occur in both adults and children. Treatment for adults is different than treatment for children. Hodgkin lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment. See the following PDQ summaries for more information: - Childhood Hodgkin Lymphoma Treatment - AIDS-Related Lymphoma Treatment Hodgkin lymphoma in pregnant women is the same as the disease in nonpregnant women of childbearing age. However, treatment is different for pregnant women. This summary includes information about treating Hodgkin lymphoma during pregnancy.
There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant.
Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four subtypes: - Nodular sclerosing Hodgkin lymphoma. - Mixed cellularity Hodgkin lymphoma. - Lymphocyte depletion Hodgkin lymphoma. - Lymphocyte-rich classical Hodgkin lymphoma. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Adult Hodgkin Lymphoma? ? | Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult Hodgkin lymphoma include the following: - Being in young or late adulthood. - Being male. - Being infected with the Epstein-Barr virus. - Having a first-degree relative (parent, brother, or sister) with Hodgkin lymphoma. Pregnancy is not a risk factor for Hodgkin lymphoma. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Adult Hodgkin Lymphoma ? | Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions. Check with your doctor if any of the following do not go away: - Painless, swollen lymph nodes in the neck, underarm, or groin. - Fever for no known reason. - Drenching night sweats. - Weight loss for no known reason. - Itchy skin. - Feeling very tired. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Adult Hodgkin Lymphoma ? | Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Sedimentation rate : A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube. The sedimentation rate is a measure of how much inflammation is in the body. A higher than normal sedimentation rate may be a sign of lymphoma or another condition. Also called erythrocyte sedimentation rate, sed rate, or ESR. - Lymph node biopsy : The removal of all or part of a lymph node. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of part of a lymph node using a wide needle. A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma. The following test may be done on tissue that was removed: - Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Adult Hodgkin Lymphoma ? | Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The patient's signs and symptoms. - The stage of the cancer. - The type of Hodgkin lymphoma. - Blood test results. - The patient's age, gender, and general health. - Whether the cancer is recurrent or progressive. For Hodgkin lymphoma during pregnancy, treatment options also depend on: - The wishes of the patient. - The age of the fetus. Adult Hodgkin lymphoma can usually be cured if found and treated early. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Adult Hodgkin Lymphoma ? | New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Adult Hodgkin Lymphoma ? | Key Points
- After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of adult Hodgkin lymphoma may include A, B, E, and S. - The following stages are used for adult Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV - Adult Hodgkin lymphoma may be grouped for treatment as follows: - Early Favorable - Early Unfavorable - Advanced
After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For adult Hodgkin lymphoma, CT scans of the neck, chest, abdomen, and pelvis are taken. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. For pregnant women with Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Stages of adult Hodgkin lymphoma may include A, B, E, and S.
Adult Hodgkin lymphoma may be described as follows: - A: The patient does not have B symptoms (fever, weight loss, or night sweats). - B: The patient has B symptoms. - E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system. - S: Cancer is found in the spleen.
The following stages are used for adult Hodgkin lymphoma:
Stage I Stage I is divided into stage I and stage IE. - Stage I: Cancer is found in one of the following places in the lymph system: - One or more lymph nodes in one lymph node group. - Waldeyer's ring. - Thymus. - Spleen. - Stage IE: Cancer is found outside the lymph system in one organ or area. Stage II Stage II is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area. Stage III Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE,S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV, the cancer: - is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or - is found outside the lymph nodes in one organ and has spread to areas far away from that organ; or - is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas.
Adult Hodgkin lymphoma may be grouped for treatment as follows:
Early Favorable Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors. Early Unfavorable Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors: - A tumor in the chest that is larger than 1/3 of the width of the chest or at least 10 centimeters. - Cancer in an organ other than the lymph nodes. - A high sedimentation rate (in a sample of blood, the red blood cells settle to the bottom of the test tube more quickly than normal). - Three or more lymph nodes with cancer. - Symptoms such as fever, weight loss, or night sweats. Advanced Advanced Hodgkin lymphoma includes some or all of the following risk factors: - Being male. - Being aged 45 years or older. - Having stage IV disease. - Having a low blood albumin (protein) level (below 4). - Having a low hemoglobin level (below 10.5). - Having a high white blood cell count (15,000 or higher). - Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count). |
Adult Hodgkin lymphoma
is a type of cancer that develops in the
lymph system. The lymph system is part of the immune system. It helps protect the body from infection and disease. The lymph system is made up of the following: Lymph tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchus, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma, including during pregnancy.
For information about Hodgkin lymphoma in children, adult non-Hodgkin lymphoma, or lymphoma in people who have acquired immunodeficiency syndrome (AIDS), see the following:
Most Hodgkin lymphomas are the classic type. When a sample of lymph node tissue is looked at under a microscope, Hodgkin lymphoma cancer cells, called Reed-Sternberg cells, may be seen. The classic type is broken down into the following four subtypes: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is rare and tends to grow slower than classic Hodgkin lymphoma. Nodular lymphocyte-predominant Hodgkin lymphoma often presents as a swollen lymph node in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at diagnosis. Treatment is often different from classic Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult
Hodgkin lymphoma include the following: These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions.
Check with your doctor if you have any of the following symptoms that do not go away: The results of the tests and procedures below also help make decisions about treatment. These tests may include: One of the following types of biopsies may be done: Other areas of the body, such as the liver, lung, bone, bone marrow, and brain, may also have a sample of tissue removed and checked by a pathologist for signs of cancer. The following test may be done on tissue that was removed: For pregnant women with Hodgkin lymphoma, imaging tests that protect the fetus from the harms of radiation are used. These include: The prognosis and treatment options depend on the following: For Hodgkin lymphoma during pregnancy, treatment options also depend on: Adult Hodgkin lymphoma can usually be cured if found and treated early. The process used to find out if cancer has spread within the lymph
system or to other parts of the body is called staging. The information gathered from the staging
process determines the stage of the
disease. It is important to know the stage to plan treatment.
The results of the tests and procedures done to diagnose and stage Hodgkin lymphoma are used to help make decisions about treatment. Cancer can spread through tissue, the lymph system, and the blood: Stage I adult Hodgkin lymphoma is divided into stages I and IE. Stage II adult Hodgkin lymphoma is divided into stages II and IIE. In stage II, the term bulky disease refers to a larger tumor mass. The size of the tumor mass that is referred to as bulky disease varies based on the type of lymphoma. In stage III adult Hodgkin lymphoma, cancer is found: In stage IV adult Hodgkin lymphoma, cancer: Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors that increase the chance that the cancer will come back after it is treated. Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors that increase the chance that the cancer will come back after it is treated: Advanced Hodgkin lymphoma is stage III or stage IV. Advanced favorable Hodgkin lymphoma means that the patient has 0–3 of the risk factors below. Advanced unfavorable Hodgkin lymphoma means that the patient has 4 or more of the risk factors below. The more risk factors a patient has, the more likely it is that the cancer will come back after it is treated: The cancer may come back in the lymph system or in other parts of the body. Different types of treatment are available for patients with adult
Hodgkin lymphoma. Some treatments
are standard (currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with
cancer. When clinical trials
show that a new treatment is better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the Hodgkin lymphoma, and the trimester of the pregnancy. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Treatment will be overseen by a medical
oncologist, a doctor who specializes in treating cancer. The
medical oncologist may refer you to other health care providers who have experience and
expertise in treating adult Hodgkin lymphoma and who specialize in certain
areas of medicine. These may include the following specialists: For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include the following: Regular follow-up by doctors who are experts in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Chemotherapy is a cancer treatment that uses one or more drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Cancer treatment using more than one chemotherapy drug is called combination chemotherapy. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second or third trimester of pregnancy. For more information, see Drugs Approved for Hodgkin Lymphoma. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Sometimes total-body irradiation is given before a stem cell transplant. Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This type of treatment can lower the amount of radiation damage to healthy tissue near a tumor such as the heart or breast. External radiation therapy is used to treat adult Hodgkin lymphoma and may also be used as palliative therapy to relieve symptoms and improve quality of life. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk of radiation exposure during fetal development. If treatment is needed right away, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover the pregnant woman's abdomen to help protect the fetus from radiation as much as possible. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. For more information, see Drugs Approved for Hodgkin Lymphoma. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. For more information, see Drugs Approved for Hodgkin Lymphoma. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Watchful waiting is
closely monitoring a patient’s condition without giving any treatment until
signs or symptoms appear or change. Active surveillance is a treatment plan that involves closely watching a patient’s condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. During active surveillance, certain exams and tests are done on a regular schedule. Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless signs or symptoms appear or change. Labor may be induced when the fetus is 32 to 36 weeks so that the mother can begin treatment. Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. When an early delivery is likely, steroids can also help the lungs of the fetus develop faster than normal. This gives babies who are born early a better chance of survival. For more information, see Drugs Approved for Hodgkin Lymphoma. Information about clinical trials is available from the
NCI website. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early favorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early unfavorable classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent classic Hodgkin lymphoma in adults may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of nodular lymphocyte–predominant Hodgkin lymphoma in adults may include the following: For information about the treatments listed below, see the Treatment Option Overview section. When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. Treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after delivery. Treatment of Hodgkin lymphoma during the second or third trimester of pregnancy may include the following: For more information from the National Cancer Institute about adult Hodgkin lymphoma, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of adult Hodgkin lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Adult Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389245] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Adult Hodgkin Lymphoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Adult Hodgkin Lymphoma ? | Key Points
- There are different types of treatment for patients with adult Hodgkin lymphoma. - Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas. - Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma. - Three types of standard treatment are used: - Chemotherapy - Radiation therapy - Surgery - For pregnant patients with Hodgkin lymphoma, treatment options also include: - Watchful waiting - Steroid therapy - New types of treatment are being tested in clinical trials. - Chemotherapy and radiation therapy with stem cell transplant - Monoclonal antibody therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with adult Hodgkin lymphoma.
Different types of treatment are available for patients with adult Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mothers wishes, the stage of the Hodgkin lymphoma, and the age of the fetus. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas.
Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer. The medical oncologist may refer you to other health care providers who have experience and expertise in treating adult Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Neurosurgeon. - Neurologist. - Rehabilitation specialist. - Radiation oncologist. - Endocrinologist. - Hematologist. - Other oncology specialists.
Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma.
Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include: - Acute myelogenous leukemia. - Cancer of the breast, bone, cervix, gastrointestinal tract, head and neck, lung, soft tissue, and thyroid. - Heart, lung, and thyroid disease. - Avascular necrosis of bone (death of bone cells caused by lack of blood flow). - Herpes zoster (shingles) or severe infection. - Depression and fatigue. - Infertility. - Hypogonadism (low levels of testosterone and estrogen). Regular follow-up by doctors who are expert in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma.
Three types of standard treatment are used:
Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug. When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second half of pregnancy. See Drugs Approved for Hodgkin Lymphoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat adult Hodgkin lymphoma. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk to the fetus. If immediate treatment is needed, the woman may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the fetus may not protect it from scattered radiation that could possibly cause cancer in the future. Surgery Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. If cancer is found, the tissue or organ is removed during the laparotomy.
For pregnant patients with Hodgkin lymphoma, treatment options also include:
Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment unless signs or symptoms appear or change. Delivery may be induced when the fetus is 32 to 36 weeks old, so that the mother can begin treatment. Steroid therapy Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. Steroids can also help the lungs of the fetus develop faster than normal. This is important when delivery is induced early. See Drugs Approved for Hodgkin Lymphoma for more information.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Adult Hodgkin Lymphoma
Early Favorable Hodgkin Lymphoma
Treatment of early favorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. - Radiation therapy alone to areas of the body with cancer or to the mantle field (neck, chest, armpits). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Early Unfavorable Hodgkin Lymphoma
Treatment of early unfavorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Advanced Hodgkin Lymphoma
Treatment of advanced Hodgkin lymphoma may include the following: - Combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III adult Hodgkin lymphoma and stage IV adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Recurrent Adult Hodgkin Lymphoma
Treatment of recurrent Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy followed by high-dose chemotherapy and stem cell transplant with or without radiation therapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer in patients older than 60 years. - Radiation therapy with or without chemotherapy. - Chemotherapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of high-dose chemotherapy and stem cell transplant. - A clinical trial of lower-dose chemotherapy and radiation therapy followed by stem cell transplant. - A clinical trial of a monoclonal antibody. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Treatment Options for Hodgkin Lymphoma During Pregnancy
Hodgkin Lymphoma During the First Trimester of Pregnancy
When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. For women who choose to continue the pregnancy, treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: - Watchful waiting when the cancer is above the diaphragm and is slow-growing. Delivery may be induced when the fetus is 32 to 36 weeks old so the mother can begin treatment. - Radiation therapy above the diaphragm. (A lead shield is used to protect the fetus from the radiation as much as possible.) - Systemic chemotherapy using one or more drugs.
Hodgkin Lymphoma During the Second Half of Pregnancy
When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after the baby is born. Treatment of Hodgkin lymphoma during the second half of pregnancy may include the following: - Watchful waiting, with plans to induce delivery when the fetus is 32 to 36 weeks old. - Systemic chemotherapy using one or more drugs. - Steroid therapy. - Radiation therapy to relieve breathing problems caused by a large tumor in the chest. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Hairy Cell Leukemia ? | Key Points
- Hairy cell leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Gender and age may affect the risk of hairy cell leukemia. - Signs and symptoms of hairy cell leukemia include infections, tiredness, and pain below the ribs. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukemia. - Certain factors affect treatment options and prognosis (chance of recovery).
Hairy cell leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).
Hairy cell leukemia is a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia because the leukemia cells look "hairy" when viewed under a microscope.
Leukemia may affect red blood cells, white blood cells, and platelets.
Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. A lymphoid stem cell becomes a lymphoblast cell and then into one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make antibodies to help fight infection. - Natural killer cells that attack cancer cells and viruses. In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. They are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia. See the following PDQ summaries for information about other types of leukemia: - Adult Acute Lymphoblastic Leukemia Treatment. - Childhood Acute Lymphoblastic Leukemia Treatment. - Chronic Lymphocytic Leukemia Treatment. - Adult Acute Myeloid Leukemia Treatment. - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment. - Chronic Myelogenous Leukemia Treatment. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Hairy Cell Leukemia? ? | Gender and age may affect the risk of hairy cell leukemia. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in older men. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Hairy Cell Leukemia ? | Signs and symptoms of hairy cell leukemia include infections, tiredness, and pain below the ribs. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions. Check with your doctor if you have any of the following: - Weakness or feeling tired. - Fever or frequent infections. - Easy bruising or bleeding. - Shortness of breath. - Weight loss for no known reason. - Pain or a feeling of fullness below the ribs. - Painless lumps in the neck, underarm, stomach, or groin. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Hairy Cell Leukemia ? | Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukemia. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as a swollen spleen, lumps, or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for cells that look "hairy," the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. - Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see what type of cell it is. This test is done to diagnose the specific type of leukemia by comparing the cancer cells to normal cells of the immune system. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - Gene mutation test: A laboratory test done on a bone marrow or blood sample to check for mutations in the BRAF gene. A BRAF gene mutation is often found in patients with hairy cell leukemia. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Hairy Cell Leukemia ? | Certain factors affect treatment options and prognosis (chance of recovery). The treatment options may depend on the following: - The number of hairy (leukemia) cells and healthy blood cells in the blood and bone marrow. - Whether the spleen is swollen. - Whether there are signs or symptoms of leukemia, such as infection. - Whether the leukemia has recurred (come back) after previous treatment. The prognosis (chance of recovery) depends on the following: - Whether the hairy cell leukemia does not grow or grows so slowly it does not need treatment. - Whether the hairy cell leukemia responds to treatment. Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Hairy Cell Leukemia ? | Key Points
- There is no standard staging system for hairy cell leukemia.
There is no standard staging system for hairy cell leukemia.
Staging is the process used to find out how far the cancer has spread. Groups are used in place of stages for hairy cell leukemia. The disease is grouped as untreated, progressive, or refractory. Untreated hairy cell leukemia The hairy cell leukemia is newly diagnosed and has not been treated except to relieve signs or symptoms such as weight loss and infections. In untreated hairy cell leukemia, some or all of the following conditions occur: - Hairy (leukemia) cells are found in the blood and bone marrow. - The number of red blood cells, white blood cells, or platelets may be lower than normal. - The spleen may be larger than normal. Progressive hairy cell leukemia In progressive hairy cell leukemia, the leukemia has been treated with either chemotherapy or splenectomy (removal of the spleen) and one or both of the following conditions occur: - There is an increase in the number of hairy cells in the blood or bone marrow. - The number of red blood cells, white blood cells, or platelets in the blood is lower than normal. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Hairy Cell Leukemia ? | New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
Hairy cell leukemia is
a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia
because the leukemia cells look "hairy" when viewed
under a microscope. Normally,
the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia.
For information about treatment of other types of leukemia, see the following PDQ summaries: Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in men. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions.
Check with your doctor if you have any of the following: The following tests and procedures may be used: The treatment options depend on the following: The prognosis depends on the following: Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission. The process used to find out if cancer has spread to other parts of the body is called staging. There is no standard staging system for hairy cell leukemia. In untreated hairy cell leukemia, some or all of the following conditions occur: Refractory hairy cell leukemia is cancer that does not respond to treatment. Recurrent hairy cell leukemia is cancer that has recurred (come back) after it has been treated. Different types of treatment are available for patients with
hairy cell leukemia. Some treatments
are standard (the currently used treatment), and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new
treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. See Drugs Approved for Hairy Cell Leukemia for more information. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Hairy Cell Leukemia for more information. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Rituximab is a type of monoclonal antibody that may be used to treat certain patients with hairy cell leukemia. Moxetumomab pasudotox-tdfk is a monoclonal antibody linked to a toxin that may be used to treat hairy cell leukemia that has relapsed. Other types of targeted therapies are being studied in the treatment of hairy cell leukemia, including: See Drugs Approved for Hairy Cell Leukemia for more information. Splenectomy is a surgical procedure to remove the spleen. Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of hairy cell leukemia may include the following: You and your doctor will discuss what treatment plan is best for you. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about hairy cell leukemia, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of hairy cell leukemia. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389248] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Hairy Cell Leukemia Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Hairy Cell Leukemia ? | Key Points
- There are different types of treatment for patients with hairy cell leukemia. - Five types of standard treatment are used: - Watchful waiting - Chemotherapy - Biologic therapy - Surgery - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with hairy cell leukemia.
Different types of treatment are available for patients with hairy cell leukemia. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Five types of standard treatment are used:
Watchful waiting Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. Biologic therapy Biologic therapy is a cancer treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent commonly used to treat hairy cell leukemia. See Drugs Approved for Hairy Cell Leukemia for more information. Surgery Splenectomy is a surgical procedure to remove the spleen. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy used to treat hairy cell leukemia. Monoclonal antibody therapy uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. A monoclonal antibody called rituximab may be used for certain patients with hairy cell leukemia. Other types of targeted therapies are being studied.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Hairy Cell Leukemia
Untreated Hairy Cell Leukemia
If the patient's blood cell counts are not too low and there are no signs or symptoms, treatment may not be needed and the patient is carefully watched for changes in his or her condition. If blood cell counts become too low or if signs or symptoms appear, initial treatment may include the following: - Chemotherapy. - Splenectomy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Progressive Hairy Cell Leukemia
Treatment for progressive hairy cell leukemia may include the following: - Chemotherapy. - Biologic therapy. - Splenectomy. - A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab). Check the list of NCI-supported cancer clinical trials that are now accepting patients with progressive hairy cell leukemia, initial treatment. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Relapsed or Refractory Hairy Cell Leukemia
Treatment of relapsed or refractory hairy cell leukemia may include the following: - Chemotherapy. - Biologic therapy. - Targeted therapy with a monoclonal antibody (rituximab). - High-dose chemotherapy. - A clinical trial of a new biologic therapy. - A clinical trial of a new targeted therapy. - A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab). Check the list of NCI-supported cancer clinical trials that are now accepting patients with refractory hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Key Points
- Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum. - Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue and are treated the same way. - Women who have a family history of ovarian cancer are at an increased risk of ovarian cancer. - Some ovarian, fallopian tube, and primary peritoneal cancers are caused by inherited gene mutations (changes). - Women with an increased risk of ovarian cancer may consider surgery to lessen the risk. - Signs and symptoms of ovarian, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen. - Tests that examine the ovaries and pelvic area are used to detect (find) and diagnose ovarian, fallopian tube, and peritoneal cancer. - Certain factors affect treatment options and prognosis (chance of recovery).
Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum.
The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones (chemicals that control the way certain cells or organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. Ovarian epithelial cancer is one type of cancer that affects the ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: - Ovarian Germ Cell Tumors - Ovarian Low Malignant Potential Tumors - Unusual Cancers of Childhood Treatment (ovarian cancer in children)
Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue and are treated the same way. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer? ? | Women who have a family history of ovarian cancer are at an increased risk of ovarian cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Women who have one first-degree relative (mother, daughter, or sister) with a history of ovarian cancer have an increased risk of ovarian cancer. This risk is higher in women who have one first-degree relative and one second-degree relative (grandmother or aunt) with a history of ovarian cancer. This risk is even higher in women who have two or more first-degree relatives with a history of ovarian cancer. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Is Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer inherited ? | Some ovarian, fallopian tube, and primary peritoneal cancers are caused by inherited gene mutations (changes). The genes in cells carry the hereditary information that is received from a persons parents. Hereditary ovarian cancer makes up about 20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These genetic tests are sometimes done for members of families with a high risk of cancer. See the following PDQ summaries for more information: - Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention - Genetics of Breast and Gynecologic Cancers (for health professionals) |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to prevent Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Women with an increased risk of ovarian cancer may consider surgery to lessen the risk. Some women who have an increased risk of ovarian cancer may choose to have a risk-reducing oophorectomy (the removal of healthy ovaries so that cancer cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the symptoms of Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Signs and symptoms of ovarian, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen. Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: - Pain, swelling, or a feeling of pressure in the abdomen or pelvis. - Vaginal bleeding that is heavy or irregular, especially after menopause. - Vaginal discharge that is clear, white, or colored with blood. - A lump in the pelvic area. - Gastrointestinal problems, such as gas, bloating, or constipation. These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Tests that examine the ovaries and pelvic area are used to detect (find) and diagnose ovarian, fallopian tube, and peritoneal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - CA 125 assay : A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level can be a sign of cancer or another condition such as endometriosis. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs in the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. Some patients may have a transvaginal ultrasound. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A very small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue is usually removed during surgery to remove the tumor. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Certain factors affect treatment options and prognosis (chance of recovery). The prognosis (chance of recovery) and treatment options depend on the following: - The type of ovarian cancer and how much cancer there is. - The stage and grade of the cancer. - Whether the patient has extra fluid in the abdomen that causes swelling. - Whether all of the tumor can be removed by surgery. - Whether there are changes in the BRCA1 or BRCA2 genes. - The patients age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Key Points
- After ovarian, fallopian tube, or peritoneal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the ovaries or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for ovarian epithelial, fallopian tube, and primary peritoneal cancer: - Stage I - Stage II - Stage III - Stage IV - Ovarian epithelial, fallopian tube, and primary peritoneal cancers are grouped for treatment as early or advanced cancer.
After ovarian, fallopian tube, or peritoneal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the ovaries or to other parts of the body.
The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.)
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer.
The following stages are used for ovarian epithelial, fallopian tube, and primary peritoneal cancer:
Stage I In stage I, cancer is found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: Cancer is found inside a single ovary or fallopian tube. - Stage IB: Cancer is found inside both ovaries or fallopian tubes. - Stage IC: Cancer is found inside one or both ovaries or fallopian tubes and one of the following is true: - cancer is also found on the outside surface of one or both ovaries or fallopian tubes; or - the capsule (outer covering) of the ovary ruptured (broke open) before or during surgery; or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, cancer is found in one or both ovaries or fallopian tubes and has spread into other areas of the pelvis, or primary peritoneal cancer is found within the pelvis. Stage II ovarian epithelial and fallopian tube cancers are divided into stage IIA and stage IIB. - Stage IIA: Cancer has spread from where it first formed to the uterus and/or the fallopian tubes and/or the ovaries. - Stage IIB: Cancer has spread from the ovary or fallopian tube to organs in the peritoneal cavity (the space that contains the abdominal organs). Stage III In stage III, cancer is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - In stage IIIA, one of the following is true: - Cancer has spread to lymph nodes in the area outside or behind the peritoneum only; or - Cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum outside the pelvis. Cancer may have spread to nearby lymph nodes. - Stage IIIB: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is 2 centimeters or smaller. Cancer may have spread to lymph nodes behind the peritoneum. - Stage IIIC: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is larger than 2 centimeters. Cancer may have spread to lymph nodes behind the peritoneum or to the surface of the liver or spleen. Stage IV In stage IV, cancer has spread beyond the abdomen to other parts of the body. Stage IV is divided into stage IVA and stage IVB. - Stage IVA: Cancer cells are found in extra fluid that builds up around the lungs. - Stage IVB: Cancer has spread to organs and tissues outside the abdomen, including lymph nodes in the groin.
Ovarian epithelial, fallopian tube, and primary peritoneal cancers are grouped for treatment as early or advanced cancer.
Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | Key Points
- There are different types of treatment for patients with ovarian epithelial cancer. - Three kinds of standard treatment are used. - Surgery - Chemotherapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Radiation therapy - Immunotherapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with ovarian epithelial cancer.
Different types of treatment are available for patients with ovarian epithelial cancer. Some treatments are standard, and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the treatment currently used as standard treatment, the new treatment may become the standard treatment. Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Three kinds of standard treatment are used.
Surgery Most patients have surgery to remove as much of the tumor as possible. Different types of surgery may include: - Hysterectomy: Surgery to remove the uterus and, sometimes, the cervix. When only the uterus is removed, it is called a partial hysterectomy. When both the uterus and the cervix are removed, it is called a total hysterectomy. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Unilateral salpingo-oophorectomy: A surgical procedure to remove one ovary and one fallopian tube. - Bilateral salpingo-oophorectomy: A surgical procedure to remove both ovaries and both fallopian tubes. - Omentectomy: A surgical procedure to remove the omentum (tissue in the peritoneum that contains blood vessels, nerves, lymph vessels, and lymph nodes). - Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib and niraparib are PARP inhibitors that may be used to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called intraperitoneal radiation therapy, in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat advanced ovarian cancer.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Stage
Early Ovarian Epithelial and Fallopian Tube Cancer
Treatment of early ovarian epithelial cancer or fallopian tube cancer may include the following: - Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope for cancer cells. Chemotherapy may be given after surgery. - Unilateral salpingo-oophorectomy may be done in certain women who wish to have children. Chemotherapy may be given after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I ovarian epithelial cancer and fallopian tube cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Advanced Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer
Treatment of advanced ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: - Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope to look for cancer cells. Surgery is followed by one of the following: - Intravenous chemotherapy. - Intraperitoneal chemotherapy. - Chemotherapy and targeted therapy (bevacizumab, olaparib, or niraparib). - Chemotherapy followed by hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. - Chemotherapy alone for patients who cannot have surgery. - A clinical trial of intraperitoneal radiation therapy, immunotherapy (vaccine therapy), or targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II ovarian epithelial cancer, stage III ovarian epithelial cancer, stage IV ovarian epithelial cancer, fallopian tube cancer and primary peritoneal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. |
The ovaries are a pair
of organs in the female reproductive
system. They are in the pelvis, one on each side of the
uterus (the hollow, pear-shaped
organ where a fetus grows). Each
ovary is about the size and shape of an almond. The ovaries make eggs and
female hormones (chemicals that
control the way certain cells or
organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium. Ovarian epithelial cancer
is one type of cancer that affects the
ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: Anything that increases your chance of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for ovarian cancer. Risk factors for ovarian cancer include the following: Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. The genes in cells carry the hereditary information
that is received from a person’s parents. Hereditary ovarian cancer makes up about
20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and
colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These
genetic tests are sometimes done for
members of families with a high risk of cancer. See the following PDQ summaries for
more information: Some women who have an increased risk of ovarian
cancer may choose to have a risk-reducing
oophorectomy (the removal of healthy ovaries so that cancer
cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.) Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible. The following tests and procedures may be used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer: The prognosis and treatment options depend on the following: The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section for tests and procedures used to diagnose and stage ovarian, fallopian tube, and peritoneal cancer.) Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. In stage I, cancer is
found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. In stage II, cancer is
found in one or both ovaries or fallopian tubes and has spread into other areas of the
pelvis, or primary peritoneal cancer is found within the pelvis. Stage II is divided
into stage IIA and stage IIB. In stage III, cancer
is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IV, cancer has spread beyond the abdomen to other
parts of the body. Stage IV is divided into stage IVA and stage IVB. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers. The cancer may come back in the same place or in other parts of the body. Persistent cancer is cancer that does not go away with treatment. Different types of treatment are available for patients with
ovarian epithelial cancer. Some
treatments are standard, and some are being tested in
clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer.
When clinical trials show that a new treatment is better than the treatment
currently used as standard
treatment, the new treatment may become the standard treatment.
Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Most patients have surgery to remove as much of the
tumor as possible. Different types
of surgery may include: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment used during surgery that is being studied for ovarian cancer. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Bevacizumab is a monoclonal antibody and angiogenesis inhibitor that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). It binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Other angiogenesis inhibitors are being studied in the treatment of advanced or recurrent ovarian cancer. Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib, rucaparib, and niraparib are PARP inhibitors that may be used as maintenance therapy to treat certain types of ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that have recurred. Veliparib is a PARP inhibitor that is being studied in combination with chemotherapy to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called
intraperitoneal radiation therapy,
in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy. Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat advanced ovarian cancer. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of early ovarian epithelial
cancer or fallopian tube cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of advanced ovarian
epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of recurrent
ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about ovarian epithelial, fallopian tube, and primary peritoneal cancer, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of ovarian epithelial, fallopian tube, and primary peritoneal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389163] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ? | New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called intraperitoneal radiation therapy, in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat advanced ovarian cancer.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
What is (are) Paranasal Sinus and Nasal Cavity Cancer ? | Key Points
- Paranasal sinus and nasal cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the paranasal sinuses and nasal cavity. - Different types of cells in the paranasal sinus and nasal cavity may become malignant. - Being exposed to certain chemicals or dust in the workplace can increase the risk of paranasal sinus and nasal cavity cancer. - Signs of paranasal sinus and nasal cavity cancer include sinus problems and nosebleeds. - Tests that examine the sinuses and nasal cavity are used to detect (find) and diagnose paranasal sinus and nasal cavity cancer. - Certain factors affect prognosis (chance of recovery) and treatment options.
Paranasal sinus and nasal cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the paranasal sinuses and nasal cavity.
Paranasal sinuses "Paranasal" means near the nose. The paranasal sinuses are hollow, air-filled spaces in the bones around the nose. The sinuses are lined with cells that make mucus, which keeps the inside of the nose from drying out during breathing. There are several paranasal sinuses named after the bones that surround them: - The frontal sinuses are in the lower forehead above the nose. - The maxillary sinuses are in the cheekbones on either side of the nose. - The ethmoid sinuses are beside the upper nose, between the eyes. - The sphenoid sinuses are behind the nose, in the center of the skull. Nasal cavity The nose opens into the nasal cavity, which is divided into two nasal passages. Air moves through these passages during breathing. The nasal cavity lies above the bone that forms the roof of the mouth and curves down at the back to join the throat. The area just inside the nostrils is called the nasal vestibule. A small area of special cells in the roof of each nasal passage sends signals to the brain to give the sense of smell. Together the paranasal sinuses and the nasal cavity filter and warm the air, and make it moist before it goes into the lungs. The movement of air through the sinuses and other parts of the respiratory system help make sounds for talking. Paranasal sinus and nasal cavity cancer is a type of head and neck cancer.
Different types of cells in the paranasal sinus and nasal cavity may become malignant.
The most common type of paranasal sinus and nasal cavity cancer is squamous cell carcinoma. This type of cancer forms in the squamous cells (thin, flat cells) lining the inside of the paranasal sinuses and the nasal cavity. Other types of paranasal sinus and nasal cavity cancer include the following: - Melanoma: Cancer that starts in cells called melanocytes, the cells that give skin its natural color. - Sarcoma: Cancer that starts in muscle or connective tissue. - Inverting papilloma: Benign tumors that form inside the nose. A small number of these change into cancer. - Midline granulomas: Cancer of tissues in the middle part of the face. | |
Who is at risk for Paranasal Sinus and Nasal Cavity Cancer? ? | Being exposed to certain chemicals or dust in the workplace can increase the risk of paranasal sinus and nasal cavity cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for paranasal sinus and nasal cavity cancer include the following: - Being exposed to certain workplace chemicals or dust, such as those found in the following jobs: - Furniture-making. - Sawmill work. - Woodworking (carpentry). - Shoemaking. - Metal-plating. - Flour mill or bakery work. - Being infected with human papillomavirus (HPV). - Being male and older than 40 years. - Smoking. | |
What are the symptoms of Paranasal Sinus and Nasal Cavity Cancer ? | Signs of paranasal sinus and nasal cavity cancer include sinus problems and nosebleeds. These and other signs and symptoms may be caused by paranasal sinus and nasal cavity cancer or by other conditions. There may be no signs or symptoms in the early stages. Signs and symptoms may appear as the tumor grows. Check with your doctor if you have any of the following: - Blocked sinuses that do not clear, or sinus pressure. - Headaches or pain in the sinus areas. - A runny nose. - Nosebleeds. - A lump or sore inside the nose that does not heal. - A lump on the face or roof of the mouth. - Numbness or tingling in the face. - Swelling or other trouble with the eyes, such as double vision or the eyes pointing in different directions. - Pain in the upper teeth, loose teeth, or dentures that no longer fit well. - Pain or pressure in the ear. | |
How to diagnose Paranasal Sinus and Nasal Cavity Cancer ? | Tests that examine the sinuses and nasal cavity are used to detect (find) and diagnose paranasal sinus and nasal cavity cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Physical exam of the nose, face, and neck: An exam in which the doctor looks into the nose with a small, long-handled mirror to check for abnormal areas and checks the face and neck for lumps or swollen lymph nodes. - X-rays of the head and neck: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are three types of biopsy: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Incisional biopsy : The removal of part of an area of tissue that doesnt look normal. - Excisional biopsy : The removal of an entire area of tissue that doesnt look normal. - Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted into the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. A special tool on the nasoscope may be used to remove samples of tissue. The tissues samples are viewed under a microscope by a pathologist to check for signs of cancer. - Laryngoscopy : A procedure to look at the larynx (voice box) for abnormal areas. A mirror or a laryngoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth to see the larynx. A special tool on the laryngoscope may be used to remove samples of tissue. The tissue samples are viewed under a microscope by a pathologist to check for signs of cancer. | |
What is the outlook for Paranasal Sinus and Nasal Cavity Cancer ? | Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Where the tumor is in the paranasal sinus or nasal cavity and whether it has spread. - The size of the tumor. - The type of cancer. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). Paranasal sinus and nasal cavity cancers often have spread by the time they are diagnosed and are hard to cure. After treatment, a lifetime of frequent and careful follow-up is important because there is an increased risk of developing a second kind of cancer in the head or neck. | |
What are the stages of Paranasal Sinus and Nasal Cavity Cancer ? | Key Points
- After paranasal sinus and nasal cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the paranasal sinuses and nasal cavity or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There is no standard staging system for cancer of the sphenoid and frontal sinuses. - The following stages are used for maxillary sinus cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV - The following stages are used for nasal cavity and ethmoid sinus cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV
After paranasal sinus and nasal cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the paranasal sinuses and nasal cavity or to other parts of the body.
The process used to find out if cancer has spread within the paranasal sinuses and nasal cavity or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an opening in the body, such as the nose or mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. Sometimes a substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if nasal cavity cancer spreads to the lung, the cancer cells in the lung are actually nasal cavity cancer cells. The disease is metastatic nasal cavity cancer, not lung cancer.
There is no standard staging system for cancer of the sphenoid and frontal sinuses.
The following stages are used for maxillary sinus cancer:
Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the innermost lining of the maxillary sinus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed in the mucous membranes of the maxillary sinus. Stage II In stage II, cancer has spread to bone around the maxillary sinus, including the roof of the mouth and the nose, but not to bone at the back of the maxillary sinus or the base of the skull. Stage III In stage III, cancer has spread to any of the following: - Bone at the back of the maxillary sinus. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. or Cancer has spread to one lymph node on the same side of the neck as the cancer and the lymph node is 3 centimeters or smaller. Cancer has also spread to any of the following: - The lining of the maxillary sinus. - Bones around the maxillary sinus, including the roof of the mouth and the nose. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. Stage IV Stage IV is divided into stage IVA, IVB, and IVC. Stage IVA In stage IVA, cancer has spread: - to one lymph node on the same side of the neck as the cancer and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the original tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the original tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. and cancer has spread to any of the following: - The lining of the maxillary sinus. - Bones around the maxillary sinus, including the roof of the mouth and the nose. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. or Cancer has spread to any of the following: - The front of the eye. - The skin of the cheek. - The base of the skull. - Behind the jaw. - The bone between the eyes. - The sphenoid or frontal sinuses. and cancer may also have spread to one or more lymph nodes 6 centimeters or smaller, anywhere in the neck. Stage IVB In stage IVB, cancer has spread to any of the following: - The back of the eye. - The brain. - The middle parts of the skull. - The nerves in the head that go to the brain. - The upper part of the throat behind the nose. - The base of the skull. and cancer may be found in one or more lymph nodes of any size, anywhere in the neck. or Cancer is found in a lymph node larger than 6 centimeters. Cancer may also be found anywhere in or near the maxillary sinus. Stage IVC In stage IVC, cancer may be anywhere in or near the maxillary sinus, may have spread to lymph nodes, and has spread to organs far away from the maxillary sinus, such as the lungs.
The following stages are used for nasal cavity and ethmoid sinus cancer:
Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the innermost lining of the nasal cavity or ethmoid sinus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and is found in only one area (of either the nasal cavity or the ethmoid sinus) and may have spread into bone. Stage II In stage II, cancer is found in two areas (of either the nasal cavity or the ethmoid sinus) that are near each other or has spread to an area next to the sinuses. Cancer may also have spread into bone. Stage III In stage III, cancer has spread to any of the following: - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. or Cancer has spread to one lymph node on the same side of the neck as the cancer and the lymph node is 3 centimeters or smaller. Cancer has also spread to any of the following: - The nasal cavity. - The ethmoid sinus. - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. Stage IV Stage IV is divided into stage IVA, IVB, and IVC. Stage IVA In stage IVA, cancer has spread: - to one lymph node on the same side of the neck as the cancer and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the original tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the original tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. and cancer has spread to any of the following: - The nasal cavity. - The ethmoid sinus. - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. or Cancer has spread to any of the following: - The front of the eye. - The skin of the nose or cheek. - Front parts of the skull. - The base of the skull. - The sphenoid or frontal sinuses. and cancer may have spread to one or more lymph nodes 6 centimeters or smaller, anywhere in the neck. Stage IVB In stage IVB, cancer has spread to any of the following: - The back of the eye. - The brain. - The middle parts of the skull. - The nerves in the head that go to the brain. - The upper part of the throat behind the nose. - The base of the skull. and cancer may be found in one or more lymph nodes of any size, anywhere in the neck. or Cancer is found in a lymph node larger than 6 centimeters. Cancer may also be found anywhere in or near the nasal cavity and ethmoid sinus. Stage IVC In stage IVC, cancer may be anywhere in or near the nasal cavity and ethmoid sinus, may have spread to lymph nodes, and has spread to organs far away from the nasal cavity and ethmoid sinus, such as the lungs. | |
What are the treatments for Paranasal Sinus and Nasal Cavity Cancer ? | Key Points
- There are different types of treatment for patients with paranasal sinus and nasal cavity cancer. - Patients with paranasal sinus and nasal cavity cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with paranasal sinus and nasal cavity cancer.
Different types of treatment are available for patients with paranasal sinus and nasal cavity cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Patients with paranasal sinus and nasal cavity cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer.
Treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. The medical oncologist works with other doctors who are experts in treating patients with head and neck cancer and who specialize in certain areas of medicine and rehabilitation. Patients who have paranasal sinus and nasal cavity cancer may need special help adjusting to breathing problems or other side effects of the cancer and its treatment. If a large amount of tissue or bone around the paranasal sinuses or nasal cavity is taken out, plastic surgery may be done to repair or rebuild the area. The treatment team may include the following specialists: - Radiation oncologist. - Neurologist. - Oral surgeon or head and neck surgeon. - Plastic surgeon. - Dentist. - Nutritionist. - Speech and language pathologist. - Rehabilitation specialist.
Three types of standard treatment are used:
Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of paranasal sinus and nasal cavity cancer. A doctor may remove the cancer and some of the healthy tissue and bone around the cancer. If the cancer has spread, the doctor may remove lymph nodes and other tissues in the neck. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. The total dose of radiation therapy is sometimes divided into several smaller, equal doses delivered over a period of several days. This is called fractionation. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat paranasal sinus and nasal cavity cancer. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. The thyroid hormone levels in the blood may be tested before and after treatment. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Head and Neck Cancer for more information. (Paranasal sinus and nasal cavity cancer is a type of head and neck cancer.)
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Stage
Stage I Paranasal Sinus and Nasal Cavity Cancer
Treatment of stage I paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity: - If cancer is in the maxillary sinus, treatment is usually surgery with or without radiation therapy. - If cancer is in the ethmoid sinus, treatment is usually radiation therapy and/or surgery. - If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) - If cancer is in the nasal cavity, treatment is usually surgery and/or radiation therapy. - If cancer is in the nasal vestibule, treatment is usually surgery or radiation therapy. - For inverting papilloma, treatment is usually surgery with or without radiation therapy. - For melanoma and sarcoma, treatment is usually surgery with or without radiation therapy and chemotherapy. - For midline granuloma, treatment is usually radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage II Paranasal Sinus and Nasal Cavity Cancer
Treatment of stage II paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity: - If cancer is in the maxillary sinus, treatment is usually high-dose radiation therapy before or after surgery. - If cancer is in the ethmoid sinus, treatment is usually radiation therapy and/or surgery. - If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) - If cancer is in the nasal cavity, treatment is usually surgery and/or radiation therapy. - If cancer is in the nasal vestibule, treatment is usually surgery or radiation therapy. - For inverting papilloma, treatment is usually surgery with or without radiation therapy. - For melanoma and sarcoma, treatment is usually surgery with or without radiation therapy and chemotherapy. - For midline granuloma, treatment is usually radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage III Paranasal Sinus and Nasal Cavity Cancer
Treatment of stage III paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity. If cancer is in the maxillary sinus, treatment may include the following: - High-dose radiation therapy before or after surgery. - A clinical trial of fractionated radiation therapy before or after surgery. If cancer is in the ethmoid sinus, treatment may include the following: - Surgery followed by radiation therapy. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) If cancer is in the nasal cavity, treatment may include the following: - Surgery and/or radiation therapy. - Chemotherapy and radiation therapy. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. For inverting papilloma, treatment is usually surgery with or without radiation therapy. For melanoma and sarcoma, treatment may include the following: - Surgery. - Radiation therapy. - Surgery, radiation therapy, and chemotherapy. For midline granuloma, treatment is usually radiation therapy. If cancer is in the nasal vestibule, treatment may include the following: - External radiation therapy and/or internal radiation therapy with or without surgery. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Stage IV Paranasal Sinus and Nasal Cavity Cancer
Treatment of stage IV paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity. If cancer is in the maxillary sinus, treatment may include the following: - High-dose radiation therapy with or without surgery. - A clinical trial of fractionated radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. If cancer is in the ethmoid sinus, treatment may include the following: - Radiation therapy before or after surgery. - Chemotherapy and radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) If cancer is in the nasal cavity, treatment may include the following: - Surgery and/or radiation therapy. - Chemotherapy and radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. For inverting papilloma, treatment is usually surgery with or without radiation therapy. For melanoma and sarcoma, treatment may include the following: - Surgery. - Radiation therapy. - Chemotherapy. For midline granuloma, treatment is usually radiation therapy. If cancer is in the nasal vestibule, treatment may include the following: - External radiation therapy and/or internal radiation therapy with or without surgery. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. | |
what research (or clinical trials) is being done for Paranasal Sinus and Nasal Cavity Cancer ? | New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. | |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies
directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles.EnlargeAnatomy of the male reproductive and urinary systems, showing the testicles, prostate, bladder, and other organs. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than Black men to have testicular cancer. Although the number of new cases of testicular cancer has doubled in the last 40 years,
the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.) Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. There is no standard or routine screening test used for early detection of testicular cancer. Most often, testicular cancer is first found by men themselves, either by chance or during self-exam. Sometimes the cancer is found by a doctor during a routine physical exam. No studies have been done to find out if testicular self-exams, regular exams by a doctor, or other screening tests in men with no symptoms would decrease the risk of dying from this disease. However, routine screening probably would not decrease the risk of dying from testicular cancer. This is partly because testicular cancer can usually be cured at any stage. Finding testicular cancer early may make it easier to treat. Patients who are diagnosed with testicular cancer that has not spread to other parts of the body may need less chemotherapy and surgery, resulting in fewer side effects. If a lump is found in the testicle by the patient or during a routine physical exam, tests may be done to check for cancer. Some tests have risks, and may cause anxiety. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about testicular cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Testicular Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389226] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Testicular Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Testicular Cancer ? | Key Points
- Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles. - Testicular cancer is the most common cancer in men aged 15 to 34 years. - Testicular cancer can usually be cured. - A condition called cryptorchidism (an undescended testicle) is a risk factor for testicular cancer.
Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles.
The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer. |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies
directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles.EnlargeAnatomy of the male reproductive and urinary systems, showing the testicles, prostate, bladder, and other organs. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than Black men to have testicular cancer. Although the number of new cases of testicular cancer has doubled in the last 40 years,
the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.) Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. There is no standard or routine screening test used for early detection of testicular cancer. Most often, testicular cancer is first found by men themselves, either by chance or during self-exam. Sometimes the cancer is found by a doctor during a routine physical exam. No studies have been done to find out if testicular self-exams, regular exams by a doctor, or other screening tests in men with no symptoms would decrease the risk of dying from this disease. However, routine screening probably would not decrease the risk of dying from testicular cancer. This is partly because testicular cancer can usually be cured at any stage. Finding testicular cancer early may make it easier to treat. Patients who are diagnosed with testicular cancer that has not spread to other parts of the body may need less chemotherapy and surgery, resulting in fewer side effects. If a lump is found in the testicle by the patient or during a routine physical exam, tests may be done to check for cancer. Some tests have risks, and may cause anxiety. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about testicular cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Testicular Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389226] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Testicular Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Testicular Cancer? ? | Testicular cancer is the most common cancer in men aged 15 to 34 years. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than black men to have testicular cancer |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies
directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles.EnlargeAnatomy of the male reproductive and urinary systems, showing the testicles, prostate, bladder, and other organs. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than Black men to have testicular cancer. Although the number of new cases of testicular cancer has doubled in the last 40 years,
the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.) Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. There is no standard or routine screening test used for early detection of testicular cancer. Most often, testicular cancer is first found by men themselves, either by chance or during self-exam. Sometimes the cancer is found by a doctor during a routine physical exam. No studies have been done to find out if testicular self-exams, regular exams by a doctor, or other screening tests in men with no symptoms would decrease the risk of dying from this disease. However, routine screening probably would not decrease the risk of dying from testicular cancer. This is partly because testicular cancer can usually be cured at any stage. Finding testicular cancer early may make it easier to treat. Patients who are diagnosed with testicular cancer that has not spread to other parts of the body may need less chemotherapy and surgery, resulting in fewer side effects. If a lump is found in the testicle by the patient or during a routine physical exam, tests may be done to check for cancer. Some tests have risks, and may cause anxiety. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about testicular cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Testicular Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389226] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Testicular Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Testicular Cancer ? | Testicular cancer can usually be cured. Although the number of new cases of testicular cancer has doubled in the last 40 years, the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.) |
Screening is looking for cancer before a person has any symptoms. This can help find cancer at an early stage. When abnormal tissue or cancer is found early, it may be easier to treat. By the time symptoms appear, cancer may have begun to spread. Scientists are trying to better understand which people are more likely to get certain types of cancer. They also study the things we do and the things around us to see if they cause cancer. This information helps doctors recommend who should be screened for cancer, which screening tests should be used, and how often the tests should be done. It is important to remember that your doctor does not necessarily think you have cancer if he or she suggests a screening test. Screening tests are given when you have no cancer symptoms. If a screening test result is abnormal, you may need to have more tests done to find out if you have cancer. These are called diagnostic tests. The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies
directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles.EnlargeAnatomy of the male reproductive and urinary systems, showing the testicles, prostate, bladder, and other organs. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than Black men to have testicular cancer. Although the number of new cases of testicular cancer has doubled in the last 40 years,
the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.) Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely. Scientists study screening tests to find those with the fewest harms and most benefits. Cancer screening trials also are meant to show whether early detection (finding cancer before it causes symptoms) helps a person live longer or decreases a person's chance of dying from the disease. For some types of cancer, the chance of recovery is better if the disease is found and treated at an early stage. There is no standard or routine screening test used for early detection of testicular cancer. Most often, testicular cancer is first found by men themselves, either by chance or during self-exam. Sometimes the cancer is found by a doctor during a routine physical exam. No studies have been done to find out if testicular self-exams, regular exams by a doctor, or other screening tests in men with no symptoms would decrease the risk of dying from this disease. However, routine screening probably would not decrease the risk of dying from testicular cancer. This is partly because testicular cancer can usually be cured at any stage. Finding testicular cancer early may make it easier to treat. Patients who are diagnosed with testicular cancer that has not spread to other parts of the body may need less chemotherapy and surgery, resulting in fewer side effects. If a lump is found in the testicle by the patient or during a routine physical exam, tests may be done to check for cancer. Some tests have risks, and may cause anxiety. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about testicular cancer screening. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Testicular Cancer Screening. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389226] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Testicular Cancer Screening (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Testicular Cancer? ? | A condition called cryptorchidism (an undescended testicle) is a risk factor for testicular cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: - Having cryptorchidism (an undescended testicle). - Having a testicle that is not normal, such as a small testicle that does not work the way it should. - Having testicular carcinoma in situ. - Being white. - Having a personal or family history of testicular cancer. - Having Klinefelter syndrome. Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely. |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied. The prostate is a
gland in the male
reproductive system. The prostate is just
below the bladder (the organ that
collects and empties urine) and in
front of the rectum (the lower part
of the intestine). It is about the
size of a walnut and surrounds part of the urethra (the tube that empties urine from the
bladder). The prostate gland produces fluid that makes up part of the semen.
EnlargeAnatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be
needed to correct it. The symptoms
of BPH or of other problems in the prostate may be
like symptoms of prostate cancer. EnlargeNormal prostate and benign prostatic hyperplasia (BPH). A normal prostate does not block the flow of urine from the bladder. An enlarged prostate presses on the bladder and urethra and blocks the flow of urine. Prostate cancer is most common in older men. In the U.S., about one out of every 8 men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Prostate cancer is rare in men younger than 50 years of age.
The chance of developing prostate cancer increases as men get older. A man whose father, brother, or son has had prostate cancer has a
higher-than-average risk of prostate cancer. Prostate cancer occurs more often in African American men than in White men. African American men with prostate cancer are more likely to die from the disease than White men with prostate cancer. The prostate needs male hormones to work the way it should. The main
male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the
body. DHT is important for normal prostate growth but can also cause the
prostate to get bigger and may play a part in the development of prostate cancer. The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied
whether the drug finasteride can prevent prostate cancer in healthy men 55
years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. In the PCPT, the men who took finasteride who did have prostate cancer had more aggressive tumors, but a follow-up analysis of the PCPT found that these men did not have more aggressive tumors. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The Selenium and Vitamin E Cancer Prevention Trial (SELECT)
studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older
(50 years of age and older for African American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about prostate cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Prostate Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389260] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Prostate Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Prostate Cancer ? | Key Points
- Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. - Prostate cancer is the second most common cancer among men in the United States.
Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate.
The prostate is a gland in the male reproductive system. The prostate is just below the bladder (the organ that collects and empties urine) and in front of the rectum (the lower part of the intestine). It is about the size of a walnut and surrounds part of the urethra (the tube that empties urine from the bladder). The prostate gland produces fluid that makes up part of the semen. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be needed to correct it. The symptoms of BPH or of other problems in the prostate may be like symptoms of prostate cancer. |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied. The prostate is a
gland in the male
reproductive system. The prostate is just
below the bladder (the organ that
collects and empties urine) and in
front of the rectum (the lower part
of the intestine). It is about the
size of a walnut and surrounds part of the urethra (the tube that empties urine from the
bladder). The prostate gland produces fluid that makes up part of the semen.
EnlargeAnatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be
needed to correct it. The symptoms
of BPH or of other problems in the prostate may be
like symptoms of prostate cancer. EnlargeNormal prostate and benign prostatic hyperplasia (BPH). A normal prostate does not block the flow of urine from the bladder. An enlarged prostate presses on the bladder and urethra and blocks the flow of urine. Prostate cancer is most common in older men. In the U.S., about one out of every 8 men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Prostate cancer is rare in men younger than 50 years of age.
The chance of developing prostate cancer increases as men get older. A man whose father, brother, or son has had prostate cancer has a
higher-than-average risk of prostate cancer. Prostate cancer occurs more often in African American men than in White men. African American men with prostate cancer are more likely to die from the disease than White men with prostate cancer. The prostate needs male hormones to work the way it should. The main
male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the
body. DHT is important for normal prostate growth but can also cause the
prostate to get bigger and may play a part in the development of prostate cancer. The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied
whether the drug finasteride can prevent prostate cancer in healthy men 55
years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. In the PCPT, the men who took finasteride who did have prostate cancer had more aggressive tumors, but a follow-up analysis of the PCPT found that these men did not have more aggressive tumors. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The Selenium and Vitamin E Cancer Prevention Trial (SELECT)
studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older
(50 years of age and older for African American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about prostate cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Prostate Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389260] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Prostate Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Prostate Cancer? ? | Prostate cancer is most common in older men. In the U.S., about one out of five men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: - Prostate Cancer Screening - Prostate Cancer Treatment |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied. The prostate is a
gland in the male
reproductive system. The prostate is just
below the bladder (the organ that
collects and empties urine) and in
front of the rectum (the lower part
of the intestine). It is about the
size of a walnut and surrounds part of the urethra (the tube that empties urine from the
bladder). The prostate gland produces fluid that makes up part of the semen.
EnlargeAnatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be
needed to correct it. The symptoms
of BPH or of other problems in the prostate may be
like symptoms of prostate cancer. EnlargeNormal prostate and benign prostatic hyperplasia (BPH). A normal prostate does not block the flow of urine from the bladder. An enlarged prostate presses on the bladder and urethra and blocks the flow of urine. Prostate cancer is most common in older men. In the U.S., about one out of every 8 men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Prostate cancer is rare in men younger than 50 years of age.
The chance of developing prostate cancer increases as men get older. A man whose father, brother, or son has had prostate cancer has a
higher-than-average risk of prostate cancer. Prostate cancer occurs more often in African American men than in White men. African American men with prostate cancer are more likely to die from the disease than White men with prostate cancer. The prostate needs male hormones to work the way it should. The main
male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the
body. DHT is important for normal prostate growth but can also cause the
prostate to get bigger and may play a part in the development of prostate cancer. The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied
whether the drug finasteride can prevent prostate cancer in healthy men 55
years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. In the PCPT, the men who took finasteride who did have prostate cancer had more aggressive tumors, but a follow-up analysis of the PCPT found that these men did not have more aggressive tumors. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The Selenium and Vitamin E Cancer Prevention Trial (SELECT)
studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older
(50 years of age and older for African American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about prostate cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Prostate Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389260] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Prostate Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to prevent Prostate Cancer ? | Key Points
- Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of prostate cancer: - Age - Family history of prostate cancer - Race - Hormones - Vitamin E - Folic acid - Dairy and calcium - The following protective factors may decrease the risk of prostate cancer: - Folate - Finasteride and Dutasteride - The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known: - Selenium and vitamin E - Diet - Multivitamins - Lycopene - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent prostate cancer are being studied in clinical trials.
Avoiding risk factors and increasing protective factors may help prevent cancer.
Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer.
The following risk factors may increase the risk of prostate cancer:
Age Prostate cancer is rare in men younger than 50 years of age. The chance of developing prostate cancer increases as men get older. Family history of prostate cancer A man whose father, brother, or son has had prostate cancer has a higher-than-average risk of prostate cancer. Race Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer. Hormones The prostate needs male hormones to work the way it should. The main male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the body. DHT is important for normal prostate growth but can also cause the prostate to get bigger and may play a part in the development of prostate cancer. Vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folic acid Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. Dairy and calcium A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer.
The following protective factors may decrease the risk of prostate cancer:
Folate Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and Dutasteride Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied whether the drug finasteride can prevent prostate cancer in healthy men 55 years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. Also, the men who took finasteride who did have prostate cancer had more aggressive tumors. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts).
The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known:
Selenium and vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older (50 years of age and older for African-American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. Diet It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Multivitamins Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Lycopene Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer.
Cancer prevention clinical trials are used to study ways to prevent cancer.
Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements.
New ways to prevent prostate cancer are being studied in clinical trials.
Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for prostate cancer prevention trials that are accepting patients. |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied. The prostate is a
gland in the male
reproductive system. The prostate is just
below the bladder (the organ that
collects and empties urine) and in
front of the rectum (the lower part
of the intestine). It is about the
size of a walnut and surrounds part of the urethra (the tube that empties urine from the
bladder). The prostate gland produces fluid that makes up part of the semen.
EnlargeAnatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be
needed to correct it. The symptoms
of BPH or of other problems in the prostate may be
like symptoms of prostate cancer. EnlargeNormal prostate and benign prostatic hyperplasia (BPH). A normal prostate does not block the flow of urine from the bladder. An enlarged prostate presses on the bladder and urethra and blocks the flow of urine. Prostate cancer is most common in older men. In the U.S., about one out of every 8 men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Prostate cancer is rare in men younger than 50 years of age.
The chance of developing prostate cancer increases as men get older. A man whose father, brother, or son has had prostate cancer has a
higher-than-average risk of prostate cancer. Prostate cancer occurs more often in African American men than in White men. African American men with prostate cancer are more likely to die from the disease than White men with prostate cancer. The prostate needs male hormones to work the way it should. The main
male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the
body. DHT is important for normal prostate growth but can also cause the
prostate to get bigger and may play a part in the development of prostate cancer. The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied
whether the drug finasteride can prevent prostate cancer in healthy men 55
years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. In the PCPT, the men who took finasteride who did have prostate cancer had more aggressive tumors, but a follow-up analysis of the PCPT found that these men did not have more aggressive tumors. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The Selenium and Vitamin E Cancer Prevention Trial (SELECT)
studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older
(50 years of age and older for African American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about prostate cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Prostate Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389260] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Prostate Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Prostate Cancer? ? | Avoiding risk factors and increasing protective factors may help prevent cancer.
Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer.
The following risk factors may increase the risk of prostate cancer:
Age Prostate cancer is rare in men younger than 50 years of age. The chance of developing prostate cancer increases as men get older. Family history of prostate cancer A man whose father, brother, or son has had prostate cancer has a higher-than-average risk of prostate cancer. Race Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer. Hormones The prostate needs male hormones to work the way it should. The main male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the body. DHT is important for normal prostate growth but can also cause the prostate to get bigger and may play a part in the development of prostate cancer. Vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folic acid Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. Dairy and calcium A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer.
The following protective factors may decrease the risk of prostate cancer:
Folate Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and Dutasteride Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied whether the drug finasteride can prevent prostate cancer in healthy men 55 years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. Also, the men who took finasteride who did have prostate cancer had more aggressive tumors. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts).
The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known:
Selenium and vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older (50 years of age and older for African-American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. Diet It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Multivitamins Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Lycopene Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. |
Cancer prevention is action taken to lower the chance of getting
cancer. By preventing cancer, the number of new cases of cancer
in a group or population is lowered. Hopefully, this will lower
the number of deaths caused by cancer. To prevent new cancers from starting, scientists look at risk
factors and protective factors. Anything that increases your
chance of developing cancer is called a cancer risk factor;
anything that decreases your chance of developing cancer is
called a cancer protective factor. Some risk factors for cancer can be avoided, but many cannot.
For example, both smoking and inheriting certain genes are
risk factors for some types of cancer, but only smoking can be
avoided. Regular exercise and a healthy diet may be protective
factors for some types of cancer. Avoiding risk factors and
increasing protective factors may lower your risk but it does
not mean that you will not get cancer. Different ways to prevent cancer are being studied. The prostate is a
gland in the male
reproductive system. The prostate is just
below the bladder (the organ that
collects and empties urine) and in
front of the rectum (the lower part
of the intestine). It is about the
size of a walnut and surrounds part of the urethra (the tube that empties urine from the
bladder). The prostate gland produces fluid that makes up part of the semen.
EnlargeAnatomy of the male reproductive and urinary systems, showing the prostate, testicles, bladder, and other organs. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be
needed to correct it. The symptoms
of BPH or of other problems in the prostate may be
like symptoms of prostate cancer. EnlargeNormal prostate and benign prostatic hyperplasia (BPH). A normal prostate does not block the flow of urine from the bladder. An enlarged prostate presses on the bladder and urethra and blocks the flow of urine. Prostate cancer is most common in older men. In the U.S., about one out of every 8 men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Prostate cancer is rare in men younger than 50 years of age.
The chance of developing prostate cancer increases as men get older. A man whose father, brother, or son has had prostate cancer has a
higher-than-average risk of prostate cancer. Prostate cancer occurs more often in African American men than in White men. African American men with prostate cancer are more likely to die from the disease than White men with prostate cancer. The prostate needs male hormones to work the way it should. The main
male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the
body. DHT is important for normal prostate growth but can also cause the
prostate to get bigger and may play a part in the development of prostate cancer. The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied
whether the drug finasteride can prevent prostate cancer in healthy men 55
years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. In the PCPT, the men who took finasteride who did have prostate cancer had more aggressive tumors, but a follow-up analysis of the PCPT found that these men did not have more aggressive tumors. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The Selenium and Vitamin E Cancer Prevention Trial (SELECT)
studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older
(50 years of age and older for African American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to
lower the risk of developing certain types of cancer. Some
cancer prevention trials are conducted with healthy people who
have not had cancer but who have an increased risk for cancer.
Other prevention trials are conducted with people who have had
cancer and are trying to prevent another cancer of the same type
or to lower their chance of developing a new type of cancer.
Other trials are done with healthy volunteers who are not known
to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to
find out whether actions people take can prevent cancer. These
may include eating fruits and vegetables, exercising, quitting
smoking, or taking certain medicines, vitamins, minerals, or
food supplements. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about prostate cancer prevention. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Screening and Prevention Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Screening and Prevention Editorial Board. PDQ Prostate Cancer Prevention. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389260] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Prostate Cancer Prevention (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Prostate Cancer ? | Cancer prevention clinical trials are used to study ways to prevent cancer.
Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements.
New ways to prevent prostate cancer are being studied in clinical trials.
Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for prostate cancer prevention trials that are accepting patients. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | Key Points
- Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas. - Pancreatic NETs may or may not cause signs or symptoms. - There are different kinds of functional pancreatic NETs. - Having certain syndromes can increase the risk of pancreatic NETs. - Different types of pancreatic NETs have different signs and symptoms. - Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs. - Other kinds of lab tests are used to check for the specific type of pancreatic NETs. - Certain factors affect prognosis (chance of recovery) and treatment options.
Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. There are two kinds of cells in the pancreas: - Endocrine pancreas cells make several kinds of hormones (chemicals that control the actions of certain cells or organs in the body), such as insulin to control blood sugar. They cluster together in many small groups (islets) throughout the pancreas. Endocrine pancreas cells are also called islet cells or islets of Langerhans. Tumors that form in islet cells are called islet cell tumors, pancreatic endocrine tumors, or pancreatic neuroendocrine tumors (pancreatic NETs). - Exocrine pancreas cells make enzymes that are released into the small intestine to help the body digest food. Most of the pancreas is made of ducts with small sacs at the end of the ducts, which are lined with exocrine cells. This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.
There are different kinds of functional pancreatic NETs.
Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: - Gastrinoma: A tumor that forms in cells that make gastrin. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. When increased stomach acid, stomach ulcers, and diarrhea are caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome. A gastrinoma usually forms in the head of the pancreas and sometimes forms in the small intestine. Most gastrinomas are malignant (cancer). - Insulinoma: A tumor that forms in cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. An insulinoma forms in the head, body, or tail of the pancreas. Insulinomas are usually benign (not cancer). - Glucagonoma: A tumor that forms in cells that make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar). A glucagonoma usually forms in the tail of the pancreas. Most glucagonomas are malignant (cancer). - Other types of tumors: There are other rare types of functional pancreatic NETs that make hormones, including hormones that control the balance of sugar, salt, and water in the body. These tumors include: - VIPomas, which make vasoactive intestinal peptide. VIPoma may also be called Verner-Morrison syndrome. - Somatostatinomas, which make somatostatin. These other types of tumors are grouped together because they are treated in much the same way. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | Who is at risk for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)? ? | Having certain syndromes can increase the risk of pancreatic NETs. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | How to diagnose Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs.
The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Chromogranin A test: A test in which a blood sample is checked to measure the amount of chromogranin A in the blood. A higher than normal amount of chromogranin A and normal amounts of hormones such as gastrin, insulin, and glucagon can be a sign of a non-functional pancreatic NET. - Abdominal CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS. - Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. - Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth, esophagus, and stomach into the first part of the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs of cancer. - Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. - Intraoperative ultrasound : A procedure that uses high-energy sound waves (ultrasound) to create images of internal organs or tissues during surgery. A transducer placed directly on the organ or tissue is used to make the sound waves, which create echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic NETs. Cells may be removed using a fine or wide needle inserted into the pancreas during an x-ray or ultrasound. Tissue may also be removed during a laparoscopy (a surgical incision made in the wall of the abdomen). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the blood. The radioactive material collects in bones where cancer cells have spread and is detected by a scanner.
Other kinds of lab tests are used to check for the specific type of pancreatic NETs.
The following tests and procedures may be used: Gastrinoma - Fasting serum gastrin test: A test in which a blood sample is checked to measure the amount of gastrin in the blood. This test is done after the patient has had nothing to eat or drink for at least 8 hours. Conditions other than gastrinoma can cause an increase in the amount of gastrin in the blood. - Basal acid output test: A test to measure the amount of acid made by the stomach. The test is done after the patient has had nothing to eat or drink for at least 8 hours. A tube is inserted through the nose or throat, into the stomach. The stomach contents are removed and four samples of gastric acid are removed through the tube. These samples are used to find out the amount of gastric acid made during the test and the pH level of the gastric secretions. - Secretin stimulation test : If the basal acid output test result is not normal, a secretin stimulation test may be done. The tube is moved into the small intestine and samples are taken from the small intestine after a drug called secretin is injected. Secretin causes the small intestine to make acid. When there is a gastrinoma, the secretin causes an increase in how much gastric acid is made and the level of gastrin in the blood. - Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS. Insulinoma - Fasting serum glucose and insulin test: A test in which a blood sample is checked to measure the amounts of glucose (sugar) and insulin in the blood. The test is done after the patient has had nothing to eat or drink for at least 24 hours. Glucagonoma - Fasting serum glucagon test: A test in which a blood sample is checked to measure the amount of glucagon in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours. Other tumor types - VIPoma - Serum VIP (vasoactive intestinal peptide) test: A test in which a blood sample is checked to measure the amount of VIP. - Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. In VIPoma, there is a lower than normal amount of potassium. - Stool analysis : A stool sample is checked for a higher than normal sodium (salt) and potassium levels. - Somatostatinoma - Fasting serum somatostatin test: A test in which a blood sample is checked to measure the amount of somatostatin in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours. - Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is the outlook for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | Certain factors affect prognosis (chance of recovery) and treatment options. Pancreatic NETs can often be cured. The prognosis (chance of recovery) and treatment options depend on the following: - The type of cancer cell. - Where the tumor is found in the pancreas. - Whether the tumor has spread to more than one place in the pancreas or to other parts of the body. - Whether the patient has MEN1 syndrome. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the stages of Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | Key Points
- The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body.
The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread.
The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following: - Whether the cancer is found in one place in the pancreas. - Whether the cancer is found in several places in the pancreas. - Whether the cancer has spread to lymph nodes near the pancreas or to other parts of the body such as the liver, lung, peritoneum, or bone.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What are the treatments for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | Key Points
- There are different types of treatment for patients with pancreatic NETs. - Six types of standard treatment are used: - Surgery - Chemotherapy - Hormone therapy - Hepatic arterial occlusion or chemoembolization - Targeted therapy - Supportive care - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed.
There are different types of treatment for patients with pancreatic NETs.
Different types of treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Six types of standard treatment are used:
Surgery An operation may be done to remove the tumor. One of the following types of surgery may be used: - Enucleation: Surgery to remove the tumor only. This may be done when cancer occurs in one place in the pancreas. - Pancreatoduodenectomy: A surgical procedure in which the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct are removed. Enough of the pancreas is left to make digestive juices and insulin. The organs removed during this procedure depend on the patient's condition. This is also called the Whipple procedure. - Distal pancreatectomy: Surgery to remove the body and tail of the pancreas. The spleen may also be removed. - Total gastrectomy: Surgery to remove the whole stomach. - Parietal cell vagotomy: Surgery to cut the nerve that causes stomach cells to make acid. - Liver resection: Surgery to remove part or all of the liver. - Radiofrequency ablation: The use of a special probe with tiny electrodes that kill cancer cells. Sometimes the probe is inserted directly through the skin and only local anesthesia is needed. In other cases, the probe is inserted through an incision in the abdomen. This is done in the hospital with general anesthesia. - Cryosurgical ablation: A procedure in which tissue is frozen to destroy abnormal cells. This is usually done with a special instrument that contains liquid nitrogen or liquid carbon dioxide. The instrument may be used during surgery or laparoscopy or inserted through the skin. This procedure is also called cryoablation. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Hepatic arterial occlusion or chemoembolization Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: - Stomach ulcers may be treated with drug therapy such as: - Proton pump inhibitor drugs such as omeprazole, lansoprazole, or pantoprazole. - Histamine blocking drugs such as cimetidine, ranitidine, or famotidine. - Somatostatin-type drugs such as octreotide. - Diarrhea may be treated with: - Intravenous (IV) fluids with electrolytes such as potassium or chloride. - Somatostatin-type drugs such as octreotide. - Low blood sugar may be treated by having small, frequent meals or with drug therapy to maintain a normal blood sugar level. - High blood sugar may be treated with drugs taken by mouth or insulin by injection.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Pancreatic Neuroendocrine Tumors
Gastrinoma
Treatment of gastrinoma may include supportive care and the following: - For symptoms caused by too much stomach acid, treatment may be a drug that decreases the amount of acid made by the stomach. - For a single tumor in the head of the pancreas: - Surgery to remove the tumor. - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid. - Surgery to remove the whole stomach (rare). - For a single tumor in the body or tail of the pancreas, treatment is usually surgery to remove the body or tail of the pancreas. - For several tumors in the pancreas, treatment is usually surgery to remove the body or tail of the pancreas. If tumor remains after surgery, treatment may include either: - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid; or - Surgery to remove the whole stomach (rare). - For one or more tumors in the duodenum (the part of the small intestine that connects to the stomach), treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - If no tumor is found, treatment may include the following: - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid. - Surgery to remove the whole stomach (rare). - If the cancer has spread to the liver, treatment may include: - Surgery to remove part or all of the liver. - Radiofrequency ablation or cryosurgical ablation. - Chemoembolization. - If cancer has spread to other parts of the body or does not get better with surgery or drugs to decrease stomach acid, treatment may include: - Chemotherapy. - Hormone therapy. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with gastrinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Insulinoma
Treatment of insulinoma may include the following: - For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor. - For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - For one large tumor in the body or tail of the pancreas, treatment is usually a distal pancreatectomy (surgery to remove the body and tail of the pancreas). - For more than one tumor in the pancreas, treatment is usually surgery to remove any tumors in the head of the pancreas and the body and tail of the pancreas. - For tumors that cannot be removed by surgery, treatment may include the following: - Combination chemotherapy. - Palliative drug therapy to decrease the amount of insulin made by the pancreas. - Hormone therapy. - Radiofrequency ablation or cryosurgical ablation. - For cancer that has spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the cancer. - Radiofrequency ablation or cryosurgical ablation, if the cancer cannot be removed by surgery. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with insulinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Glucagonoma
Treatment may include the following: - For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor. - For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - For more than one tumor in the pancreas, treatment is usually surgery to remove the tumor or surgery to remove the body and tail of the pancreas. - For tumors that cannot be removed by surgery, treatment may include the following: - Combination chemotherapy. - Hormone therapy. - Radiofrequency ablation or cryosurgical ablation. - For cancer that has spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the cancer. - Radiofrequency ablation or cryosurgical ablation, if the cancer cannot be removed by surgery. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with glucagonoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Other Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)
For VIPoma, treatment may include the following: - Fluids and hormone therapy to replace fluids and electrolytes that have been lost from the body. - Surgery to remove the tumor and nearby lymph nodes. - Surgery to remove as much of the tumor as possible when the tumor cannot be completely removed or has spread to distant parts of the body. This is palliative therapy to relieve symptoms and improve the quality of life. - For tumors that have spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the tumor. - Radiofrequency ablation or cryosurgical ablation, if the tumor cannot be removed by surgery. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. For somatostatinoma, treatment may include the following: - Surgery to remove the tumor. - For cancer that has spread to distant parts of the body, surgery to remove as much of the cancer as possible to relieve symptoms and improve quality of life. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: - Surgery to remove the tumor. - For cancer that has spread to distant parts of the body, surgery to remove as much of the cancer as possible or hormone therapy to relieve symptoms and improve quality of life. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with islet cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Recurrent or Progressive Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)
Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: - Surgery to remove the tumor. - Chemotherapy. - Hormone therapy. - Targeted therapy. - For liver metastases: - Regional chemotherapy. - Hepatic arterial occlusion or chemoembolization, with or without systemic chemotherapy. - A clinical trial of a new therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent islet cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. |
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. EnlargeAnatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs. There are two kinds of cells in the pancreas: This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment (Adult) for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. Pancreatic NETs may be functional or nonfunctional: Most pancreatic NETs are functional tumors. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: Anything that increases your risk of getting a disease is called
a risk factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will
not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs. Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause: Too much insulin may cause: Too much glucagon may cause: Too much vasoactive intestinal peptide (VIP) may cause: Too much somatostatin may cause: A pancreatic NET may also make too much adrenocorticotropic hormone (ACTH) and cause Cushing syndrome. Signs and symptoms of Cushing syndrome include the following: The treatment of pancreatic NETs that make too much ACTH and Cushing syndrome are not discussed in this summary. The following tests and procedures may be used: The following tests and procedures may be used: Gastrinoma Insulinoma Glucagonoma Other tumor types Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
Cancer can spread through tissue, the lymph system, and the blood: When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer. The tumors may come back in the pancreas or in other parts of the body.
Different types of
treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some
treatments are standard (the currently used treatment), and
some are being tested in clinical trials. A treatment clinical
trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients
with cancer. When clinical trials show that a new treatment is
better than the standard treatment, the new treatment may
become the standard treatment. Patients may want to think about
taking part in a clinical trial. Some clinical trials are open
only to patients who have not started treatment. An operation may be done to remove the tumor. One of the following types of surgery may be used: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver.
The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: Information about clinical trials is available from the
NCI website. For information about side effects caused by treatment for cancer, see our Side Effects page. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of gastrinoma may include supportive care and the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of insulinoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section.
Treatment may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. For VIPoma, treatment may include the following: For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about the treatment of pancreatic neuroendocrine tumors (islet cell tumors). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389340] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | what research (or clinical trials) is being done for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ? | New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. |
Plasma cells develop from B lymphocytes (B cells), a type of white blood cell that is made in the bone marrow. Normally, when bacteria or viruses enter the body, some of the B cells will change into plasma cells. The plasma cells make antibodies to fight bacteria and viruses, to stop infection and disease.EnlargeMultiple myeloma. Multiple myeloma cells are abnormal plasma cells (a type of white blood cell) that build up in the bone marrow and form tumors in many bones of the body. Normal plasma cells make antibodies to help the body fight infection and disease. As the number of multiple myeloma cells increases, more antibodies are made. This can cause the blood to thicken and keep the bone marrow from making enough healthy blood cells. Multiple myeloma cells also damage and weaken the bone. Plasma cell neoplasms are diseases in which abnormal plasma cells or cells form tumors in the bones or soft tissues of the body. The plasma cells also make an antibody protein, called M protein, that is not needed by the body and does not help fight infection. These antibody proteins build up in the bone marrow and can cause the blood to thicken or can damage the kidneys. Monoclonal gammopathy of undetermined significance (MGUS) is not cancer but can become cancer.
The following types of plasma cell neoplasms are cancer: Plasma cell neoplasms include the following: In this type of plasma cell neoplasm, less than 10% of the bone marrow is made up of abnormal plasma cells and there is no cancer. The abnormal plasma cells make M protein, which is sometimes found during a routine blood or urine test. In most patients, the amount of M protein stays the same and there are no signs, symptoms, or health problems. In some patients, MGUS may later become a more serious condition, such as amyloidosis, or cause problems with the kidneys, heart, or nerves. MGUS can also become cancer, such as multiple myeloma, lymphoplasmacytic lymphoma, or chronic lymphocytic leukemia. In this type of plasma cell neoplasm, the abnormal plasma cells (myeloma cells) are in one place and form one tumor, called a plasmacytoma. Sometimes plasmacytoma can be cured. There are two types of plasmacytoma. Signs and symptoms depend on where the tumor is. In multiple myeloma, abnormal plasma cells (myeloma cells) build up in the bone marrow and form tumors in many bones of the body. These tumors may keep the bone marrow from making enough healthy blood cells. Normally, the bone marrow makes stem cells (immature cells) that become three types of mature blood cells: As the number of myeloma cells increases, fewer red blood cells, white blood cells, and platelets are made. The myeloma cells also damage and weaken the bone. Sometimes multiple myeloma does not cause any signs or symptoms. This is called smoldering multiple myeloma. It may be found when a blood or urine test is done for another condition. Signs and symptoms may be caused by multiple myeloma or other conditions. Check with your doctor if you have any of the following: A tumor can damage the bone and cause hypercalcemia (too much calcium in the blood). This can affect many organs in the body, including the kidneys, nerves, heart, muscles, and digestive tract, and cause serious health problems. Hypercalcemia may cause the following signs and symptoms:
In rare cases, multiple myeloma can cause peripheral nerves (nerves that are not in the brain or spinal cord) and organs to fail. This may be caused by a condition called amyloidosis. Antibody proteins build up and stick together in peripheral nerves and organs, such as the kidney and heart. This can cause the nerves and organs to become stiff and unable to work the way they should. Amyloidosis may cause the following signs and symptoms: Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Plasma cell neoplasms are most common in people who are middle aged or older. For multiple myeloma and plasmacytoma, other risk factors include the following: Studies about how racial, social, and financial factors affect access to treatment and rates of plasma cell neoplasms are ongoing. The following tests and procedures may be used: The prognosis depends on the following: Treatment options depend on the following: The process used to find out the amount of cancer in the body is called staging. It is important to know
the stage in order to plan treatment. The following tests and
procedures may be used to find out how much cancer is in the body:
Beta-2-microglobulin and albumin are found in the blood. Beta-2-microglobulin is a protein found on plasma cells. Albumin makes up the biggest part of the blood plasma. It keeps fluid from leaking out of blood vessels. It also brings nutrients to tissues, and carries hormones, vitamins, drugs, and other substances, such as calcium, all through the body. In the blood of patients with multiple myeloma, the amount of beta-2-microglobulin is increased and the amount of albumin is decreased. In stage I multiple myeloma, the blood levels are as follows: In stage II multiple myeloma, the blood levels are in between the levels for stage I and stage III. In stage III multiple myeloma, the blood level of beta-2-microglobulin is 5.5 mg/L or higher and the patient also has one of the following: Plasma cell neoplasms are called refractory when the number of plasma cells keeps going up even though treatment is given. Plasma cell neoplasms are called relapsed when they have come back after treatment. Different types of treatments are available for patients with plasma cell neoplasms. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms. Corticosteroids are steroids that have antitumor effects in multiple myeloma.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Several types of targeted therapy may be used to treat multiple myeloma and other plasma cell neoplasms. There are different types of targeted therapy: See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms. High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient (autologous) or a donor (allogeneic) and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. EnlargeStem cell transplant. (Step 1): Blood is taken from a vein in the arm of the donor. The patient or another person may be the donor. The blood flows through a machine that removes the stem cells. Then the blood is returned to the donor through a vein in the other arm. (Step 2): The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown). (Step 3): The patient receives stem cells through a catheter placed into a blood vessel in the chest. Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biologic therapy. See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Surgery to remove the tumor may be done. After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Clinical trials are studying different combinations of immunotherapy, chemotherapy, steroid therapy, and drugs. New treatment regimens using selinexor are also being studied. For information about side effects caused by treatment for cancer, see our Side Effects page. This therapy controls problems or side effects caused by the disease or its treatment, and improves quality of life. Supportive care is given to treat problems caused by multiple myeloma and other plasma cell neoplasms. Supportive care may include the following:
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of monoclonal gammopathy of undetermined significance (MGUS) is usually watchful waiting. Regular blood tests to check the level of M protein in the blood and physical exams to check for signs or symptoms of cancer will be done. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of isolated plasmacytoma of bone is usually radiation therapy to the bone lesion. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of extramedullary plasmacytoma may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Patients without signs or symptoms may not need treatment. These patients can have watchful waiting until signs or symptoms appear. When signs or symptoms appear, there are two categories for patients receiving treatment: Patients younger than 65 years are usually considered younger and fit. Patients older than 75 years are usually not eligible for a stem cell transplant. For patients between the ages of 65 and 75 years, fitness is determined by their overall health and other factors. The treatment of multiple myeloma is usually done in phases: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For information about the treatments listed below, see the Treatment Option Overview section. Treatment of relapsed or refractory multiple myeloma may include the following: Drugs used may include the following: Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available. For more information from the National Cancer Institute about multiple myeloma and other plasma cell neoplasms, see the following: For general cancer information and other resources from the National Cancer Institute, see the following: Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish. PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH. This PDQ cancer information summary has current information about treatment of plasma cell neoplasms (including multiple myeloma). It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care. Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is: PDQ® Adult Treatment Editorial Board. PDQ Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389437] Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us. If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.” Want to use this content on your website or other digital platform? Our syndication services page shows you how. | What is (are) Plasma Cell Neoplasms (Including Multiple Myeloma) ? | Key Points
- Plasma cell neoplasms are diseases in which the body makes too many plasma cells. - Plasma cell neoplasms can be benign (not cancer) or malignant (cancer). - There are several types of plasma cell neoplasms. - Monoclonal gammopathy of undetermined significance (MGUS) - Plasmacytoma - Multiple myeloma - Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis. - Age can affect the risk of plasma cell neoplasms. - Tests that examine the blood, bone marrow, and urine are used to detect (find) and diagnose multiple myeloma and other plasma cell neoplasms. - Certain factors affect prognosis (chance of recovery) and treatment options.
Plasma cell neoplasms are diseases in which the body makes too many plasma cells.
Plasma cells develop from B lymphocytes (B cells), a type of white blood cell that is made in the bone marrow. Normally, when bacteria or viruses enter the body, some of the B cells will change into plasma cells. The plasma cells make antibodies to fight bacteria and viruses, to stop infection and disease. Plasma cell neoplasms are diseases in which abnormal plasma cells or myeloma cells form tumors in the bones or soft tissues of the body. The plasma cells also make an antibody protein, called M protein, that is not needed by the body and does not help fight infection. These antibody proteins build up in the bone marrow and can cause the blood to thicken or can damage the kidneys.
Plasma cell neoplasms can be benign (not cancer) or malignant (cancer).
Monoclonal gammopathy of undetermined significance (MGUS) is not cancer but can become cancer. The following types of plasma cell neoplasms are cancer: - Lymphoplasmacytic lymphoma. (See Adult Non-Hodgkin Lymphoma Treatment for more information.) - Plasmacytoma. - Multiple myeloma.
There are several types of plasma cell neoplasms.
Plasma cell neoplasms include the following: Monoclonal gammopathy of undetermined significance (MGUS) In this type of plasma cell neoplasm, less than 10% of the bone marrow is made up of abnormal plasma cells and there is no cancer. The abnormal plasma cells make M protein, which is sometimes found during a routine blood or urine test. In most patients, the amount of M protein stays the same and there are no signs, symptoms, or health problems. In some patients, MGUS may later become a more serious condition, such as amyloidosis, or cause problems with the kidneys, heart, or nerves. MGUS can also become cancer, such as multiple myeloma, lymphoplasmacytic lymphoma, or chronic lymphocytic leukemia. Plasmacytoma In this type of plasma cell neoplasm, the abnormal plasma cells (myeloma cells) are in one place and form one tumor, called a plasmacytoma. Sometimes plasmacytoma can be cured. There are two types of plasmacytoma. - In isolated plasmacytoma of bone, one plasma cell tumor is found in the bone, less than 10% of the bone marrow is made up of plasma cells, and there are no other signs of cancer. Plasmacytoma of the bone often becomes multiple myeloma. - In extramedullary plasmacytoma, one plasma cell tumor is found in soft tissue but not in the bone or the bone marrow. Extramedullary plasmacytomas commonly form in tissues of the throat, tonsil, and paranasal sinuses. Signs and symptoms depend on where the tumor is. - In bone, the plasmacytoma may cause pain or broken bones. - In soft tissue, the tumor may press on nearby areas and cause pain or other problems. For example, a plasmacytoma in the throat can make it hard to swallow. Multiple myeloma In multiple myeloma, abnormal plasma cells (myeloma cells) build up in the bone marrow and form tumors in many bones of the body. These tumors may keep the bone marrow from making enough healthy blood cells. Normally, the bone marrow makes stem cells (immature cells) that become three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to help prevent bleeding. As the number of myeloma cells increases, fewer red blood cells, white blood cells, and platelets are made. The myeloma cells also damage and weaken the bone. Sometimes multiple myeloma does not cause any signs or symptoms. This is called smoldering multiple myeloma. It may be found when a blood or urine test is done for another condition. Signs and symptoms may be caused by multiple myeloma or other conditions. Check with your doctor if you have any of the following: - Bone pain, especially in the back or ribs. - Bones that break easily. - Fever for no known reason or frequent infections. - Easy bruising or bleeding. - Trouble breathing. - Weakness of the arms or legs. - Feeling very tired. A tumor can damage the bone and cause hypercalcemia (too much calcium in the blood). This can affect many organs in the body, including the kidneys, nerves, heart, muscles, and digestive tract, and cause serious health problems. Hypercalcemia may cause the following signs and symptoms: - Loss of appetite. - Nausea or vomiting. - Feeling thirsty. - Frequent urination. - Constipation. - Feeling very tired. - Muscle weakness. - Restlessness. - Confusion or trouble thinking.
Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis.
In rare cases, multiple myeloma can cause peripheral nerves (nerves that are not in the brain or spinal cord) and organs to fail. This may be caused by a condition called amyloidosis. Antibody proteins build up and stick together in peripheral nerves and organs, such as the kidney and heart. This can cause the nerves and organs to become stiff and unable to work the way they should. Amyloidosis may cause the following signs and symptoms: - Feeling very tired. - Purple spots on the skin. - Enlarged tongue. - Diarrhea. - Swelling caused by fluid in your body's tissues. - Tingling or numbness in your legs and feet. |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.