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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTg0MzMyLW92ZXJ2aWV3\nDrugs & Diseases > Gastroenterology\nGastrinoma\nAuthor: Jennifer Lynn Bonheur, MD; Chief Editor: BS Anand, MD more...\nSections Gastrinoma\nLong-Term Monitoring\nH2-receptor antagonists\nChemotherapeutic agents\nA gastrinoma is a gastrin-secreting tumor that can occur in the pancreas, although it is most commonly found in the duodenum. [1, 2] Duodenal wall gastrinomas have been identified in 40-50% of patients. These duodenal wall tumors are frequently small and multiple. Sporadic tumors occurring in the pancreas tend to be solitary and have a greater malignant potential as compared to duodenal gastrinomas. [3]\nMore than 80% of gastrinomas arise within the triangle defined as the confluence of the cystic and common bile duct superiorly, the second and third portions of the duodenum inferiorly, and the neck and body of the pancreas medially.\nRarely, primary tumors also occur in a variety of ectopic sites, including the body of the stomach, jejunum, peripancreatic lymph nodes, [1] splenic hilum, omentum, liver, gallbladder, common bile duct, and the ovary.\nOver 50% of gastrinomas are malignant and can metastasize to the regional lymph nodes and the liver. One fourth of gastrinomas are related to multiple endocrine neoplasia (MEN) type I and are associated with hyperparathyroidism and pituitary adenomas. These MEN I associated tumors have been observed to occur at an earlier age than sporadic tumors and often follow a more benign course.\nThe triad of nonbeta islet cell tumors of the pancreas (gastrinomas), hypergastrinemia, and severe ulcer disease was described by Zollinger and Ellison in 1955, hence the eponym Zollinger-Ellison syndrome (ZES).\nEnormous secretion of gastrin from the tumor cells leads to hyperplasia of the fundic parietal cells and increased basal acid secretion. This results in severe ulcer disease. Ulceration might even extend into the small intestine. The acidic content of the small intestine causes the release of secretin, which is responsible for the diarrhea, in part, caused by the outpouring of water and bicarbonate from the pancreas and small intestine.\nA small study by Kohan et al describes duodenal bulb mucosa with hypertrophic heterotopia in patients with Zollinger-Ellison syndrome (ZES). [4] The investigators evaluated the incidence of duodenal gastric oxyntic hypertrophic heterotopia in 7 patients with ZES over an average period of 5 years. The patients underwent endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa every 3-6 months.\nOf the 7 patients with ZES and an intact stomach and duodenum, 2 had no evidence of duodenal mucosal hypertrophic heterotopia, but 5 did, in whom bowel mucosal biopsies revealed patchy replacement of the surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria. [4] In addition, patients with gastric oxyntic hypertrophic heterotopia had higher levels of average serum gastrin and basal acid output levels than those without gastric oxyntic hypertrophic heterotopia, a finding that Kohan et al suggest that not only could the hypertrophic and heterotopic gastric mucosa result from increased gastrin levels but that an increased incidence of postbulbar ulcers may result in affected patients. [4]\nUnited States data\nGastric carcinoids or neuroendocrine tumors are reported to account for an estimated 0.6-2% of detected gastric polyps. [5] In the United States, the incidence of these tumors has greatly increased over the past several decades. [5]\nThe true incidence of Zollinger-Ellison syndrome (ZES) is not known. ZES constitutes 0.1% or more of the cases of peptic ulcer disease. Although rare, gastrinomas are the most common pancreatic islet cell tumors.\nIn a 2013 German epidemiologic review of 2 time periods (1976-1988 and 1998-2006) comprising more than 10.8 million people, there was an approximately 5-fold increase in the cases of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) between 1976 and 2006, accompanied by a change in the anatomic distribution. [6]\nSex- and age-related demographics\nGastrinomas are more common in males than in females, with ratios from 1.5:1 to 2:1.\nAlthough gastrinomas can occur at any age, the initial clinical manifestation usually appears in people aged 30-50 years.\nPatients with hepatic metastases may have a remaining life span of less than 1 year; the 5-year survival rate is 20-30%.\nIn patients with localized disease or metastasis to local lymph nodes without liver metastasis, the 5-year survival rate may be 90%.\nSurgical resection of localized disease leads to a complete cure without any recurrence in 20-25% of patients with gastrinomas.\nMortality/Morbidity\nWith the advent of anti-ulcer medications, the number of deaths secondary to ulcer complications has decreased significantly. The primary determinants of survival for patients with gastrinomas are the size of the primary tumor and the occurrence of tumor metastasis.\nClinical Presentation\nHarper S, Carroll RW, Frilling A, Wickremesekera SK, Bann S. Primary lymph node gastrinoma: 2 cases and a review of the literature. J Gastrointest Surg. 2015 Apr. 19(4):651-5. [Medline].\nDoi R. Determinants of surgical resection for pancreatic neuroendocrine tumors. J Hepatobiliary Pancreat Sci. 2015 Aug. 22(8):610-7. [Medline].\nRuiz-Tovar J, Priego P, Martinez-Molina E, et al. Pancreatic neuroendocrine tumours. Clin Transl Oncol. 2008 Aug. 10(8):493-7. [Medline].\nKohan E, Oh D, Wang H, et al. Duodenal bulb mucosa with hypertrophic gastric oxyntic heterotopia in patients with Zollinger Ellison syndrome. Diagn Ther Endosc. 2009. 2009:298381. [Medline]. [Full Text].\nBasuroy R, Srirajaskanthan R, Prachalias A, Quaglia A, Ramage JK. Review article: the investigation and management of gastric neuroendocrine tumours. Aliment Pharmacol Ther. 2014 May. 39(10):1071-84. [Medline].\nScherubl H, Streller B, Stabenow R, et al. Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: epidemiological changes in Germany. World J Gastroenterol. 2013 Dec 21. 19(47):9012-9. [Medline]. [Full Text].\nRosentraeger MJ, Garbrecht N, Anlauf M, et al. Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior. Virchows Arch. 2016 Mar. 468(3):277-87. [Medline].\nRehfeld JF, Bardram L, Hilsted L, Goetze JP. An evaluation of chromogranin A versus gastrin and progastrin in gastrinoma diagnosis and control. Biomark Med. 2014 Apr. 8(4):571-80. [Medline].\nModlin IM, Aslanian H, Bodei L, Drozdov I, Kidd M. A PCR blood test outperforms chromogranin A in carcinoid detection and is unaffected by proton pump inhibitors. Endocr Connect. 2014 Dec. 3(4):215-23. [Medline].\nPoitras P, Gingras MH, Rehfeld JF. The Zollinger-Ellison syndrome: dangers and consequences of interrupting antisecretory treatment. Clin Gastroenterol Hepatol. 2012 Feb. 10(2):199-202. [Medline].\nGranberg D, Jacobsson H, Oberg K, Gustavsson J, Lehtihet M. Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report. Digestion. 2008. 77(2):92-5. [Medline].\nVillard L, Romer A, Marincek N, et al. Cohort study of somatostatin-based radiopeptide therapy with [(90)Y-DOTA]-TOC versus [(90)Y-DOTA]-TOC plus [(177)Lu-DOTA]-TOC in neuroendocrine cancers. J Clin Oncol. 2012 Apr 1. 30(10):1100-6. [Medline].\nDumont RA, Seiler D, Marincek N, et al. Survival after somatostatin based radiopeptide therapy with (90)Y-DOTATOC vs. (90)Y-DOTATOC plus (177)Lu-DOTATOC in metastasized gastrinoma. Am J Nucl Med Mol Imaging. 2015. 5(1):46-55. [Medline].\nFendrich V, Bartsch DK. Surgical treatment of gastrointestinal neuroendocrine tumors. Langenbecks Arch Surg. 2011 Mar. 396(3):299-311. [Medline].\nFernandez Ranvier GG, Shouhed D, Inabnet WB 3rd. Minimally invasive techniques for resection of pancreatic neuroendocrine tumors. Surg Oncol Clin N Am. 2016 Jan. 25(1):195-215. [Medline].\nZaidi MY, Lopez-Aguiar AG, Poultsides GA, et al, for the US Neuroendocrine Tumor Study Group. The impact of failure to achieve symptom control after resection of functional neuroendocrine tumors: An 8-institution study from the US Neuroendocrine Tumor Study Group. J Surg Oncol. 2019 Jan. 119(1):5-11. [Medline].\nHall NC, Nichols SD, Povoski SP, et al. Intraoperative use of a portable Large Field Of View Gamma Camera and Handheld Gamma Detection Probe for radioguided localization and prediction of complete surgical resection of gastrinoma: proof of concept. J Am Coll Surg. 2015 Aug. 221(2):300-8. [Medline].\nGuarnotta V, Martini C, Davi MV, et al, for the NIKE group. The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma?. Endocrine. 2018 Apr. 60(1):15-27. [Medline].\nCho MS, Kasi A. Zollinger Ellison Syndrome. StatPearls. 2019 Apr 4. [Medline]. [Full Text].\nJennifer Lynn Bonheur, MD Attending Physician, Division of Gastroenterology, Lenox Hill Hospital\nJennifer Lynn Bonheur, MD is a member of the following medical societies: American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, New York Society for Gastrointestinal Endoscopy, New York Academy of Sciences, Sigma Xi\nSenthil Nachimuthu, MD, FACP\nSenthil Nachimuthu, MD, FACP is a member of the following medical societies: American College of Physicians\nBS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine\nBS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy\nManoop S Bhutani, MD Professor, Co-Director, Center for Endoscopic Research, Training and Innovation (CERTAIN), Director, Center for Endoscopic Ultrasound, Department of Medicine, Division of Gastroenterology, University of Texas Medical Branch; Director, Endoscopic Research and Development, The University of Texas MD Anderson Cancer Center\nManoop S Bhutani, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Institute of Ultrasound in Medicine, American Society for Gastrointestinal Endoscopy\nSimmy Bank, MD Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine\nencoded search term (Gastrinoma) and Gastrinoma\nNeuroendocrine Tumors Guidelines\nPancreatic Neuroendocrine (Islet Cell) Tumor Imaging\nThymic Tumors\n5-Hydroxyindoleacetic Acid (5-HIAA)\nPathologic Findings in Small Cell Bladder Carcinoma\nOctreotide Scintigraphy\nUpdate on Neuroendocrine Carcinomas of the Larynx\nWhat Is the Appropriate Management of Nonfunctioning Pancreatic Neuroendocrine Tumours Disclosed on Screening in Adult Patients With Multiple Endocrine Neoplasia Type 1?\nFamily History Is Never 'Noncontributory'\nOn Strike or Working Overtime: 12 Endocrine Emergencies\nAccording to Oncologist/Hematologists\nHair Dyes Linked to Elevated Breast Cancer Risk\n'Truly Amazing': Huge Change in Melanoma Prognosis\nConfessions of a 'High-Yield' Doctor\nDeficiencies at MD Anderson Posed 'Jeopardy' to Patients\n'Remarkable' New Data on Menopausal Hormone Therapy\n2002 2500010-overview Diseases & Conditions\nDiseases & Conditions Neuroendocrine Tumors Guidelines\n2001 /viewarticle/921196 Journal Article\nJournal Article Update on Neuroendocrine Carcinomas of the Larynx\n2010 netspot-gallium-ga-68-dotatate-1000115 Drugs\nDrugs gallium Ga 68 dotatate\nJournal Article What Is the Appropriate Management of Nonfunctioning Pancreatic Neuroendocrine Tumours Disclosed on Screening in Adult Patients With Multiple Endocrine Neoplasia Type 1?"