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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly93d3cubWVkc2NhcGUuY29tL2Fuc3dlcnMvMTgyMDA2LTE4MDUzNi93aGF0LWlzLXRoZS1yZWxhdGlvbnNoaXAtYmV0d2Vlbi1wZXV0ei1qZWdoZXJzLXN5bmRyb21lLXBqcy1hbmQtY2FuY2Vy\nWhat is the relationship between Peutz-Jeghers syndrome (PJS) and cancer?\nPeutz-Jeghers syndrome (PJS) entails a significant overall increased lifetime risk of intestinal and extraintestinal malignancy (see the Table below; see also the Approach Considerations section), as well as an increased risk of malignancy in younger individuals. The cancer risk increases with age: 1-2% risk by age 20 years, over 30% by age 50 years, and more than 80% by age 70 years. [2] Small bowel adenocarcinoma has been diagnosed in a child as young as 13 years, [48] with patients aged through 30 years harboring a 5% cumulative risk of cancer that rises to 85% by age 70 years. The overall relative risk for cancer is greater in females than in males, and it is greatest for gastrointestinal, pancreatic, and gynecologic-cervical cancers. [2, 49]\nThe increased risk for malignancies was also documented in another study. A systematic review that included 20 studies and 1 meta-analysis, comprising 1644 patients found 21% (n = 349) of patients developed 384 malignancies. [50] The average age for malignancy was 24 years. The most common malignancy was colorectal cancer, followed by breast and small bowel cancer as well as other less common gastrointestinal malignancies. [50]\nPatients with Peutz-Jeghers syndrome appear to have an increased risk for pancreatic-biliary cancer. [51] In a study by Korsse et al that included 144 patients with Peutz-Jeghers syndrome from 61 families, 7 patients (5%) developed pancreatic cancer (median age, 54 y), and 2 patients (3%) each developed distal bile duct cancer or ampullary cancer (median age, 55 y). There was a 26% cumulative risk and 76 relative risk for pancreatic cancer at age 70 years; the cumulative risk for pancreatic-biliary cancer was 32% at age 70 years, with a relative risk of 96 (P< 0.001). [51]\nTable. Risk Ratio Compared to the General Population, Lifetime Frequency, Mean Age, and Age Range of Malignancies Related to Peutz-Jeghers Syndrome [5] (Open Table in a new window)\nRisk Ratio\nFrequency, %\nMean Age (Range), Years\n30.1 (10-61)\n8.6 (3-20)\n37 (9-48)\nOvary\nIn addition, other reproductive site cancers have been associated with Peutz-Jeghers syndrome, including adenoma malignum of the cervix, Sertoli cell tumors, and sex cord tumors with annular tubules. [52, 53, 54]\nBreast cancer in patients with Peutz-Jeghers syndrome significant increase, 8% at age 40 and 31% at age 60 [54] and it may also be bilateral. [7] Owing to the increased risk of pancreatic adenocarcinoma in Peutz-Jeghers syndrome, screening with endoscopic ultrasonography has emerged as a relatively new tool for early diagnosis. [55, 56]\nMore recent data shows the following percentage lifetime risk for cancers by site in individuals with Peutz-Jeghers syndrome [116] :\nStomach: 29%\nSmall intestine: 13%\nColon: 39%\nPancreas: 11-36%\nLung: 15-17%\nBreast: 45-50%\nUterus: 9%\nOvary: 18-21%\nCervix: 10%\nThyroid papillary malignancy [1] as well as sinonasal adenocarcinoma [57] have also been reported in patients with Peutz-Jeghers.\nTable. Risk Ratio Compared to the General Population, Lifetime Frequency, Mean Age, and Age Range of Malignancies Related to Peutz-Jeghers Syndrome [5]"