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Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.
About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.
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Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers in the stomach or the first part of the small intestine called the duodenum.
Factors that may increase your chance of Zollinger-Ellison syndrome include:
In many people, Zollinger-Ellison syndrome causes symptoms similar to an ulcer or gastroesophageal reflux disease (GERD).
Symptoms may include:
You will be asked about your symptoms and medical history. A physical exam will be done.
Tests may include:
Imaging tests may help your doctor to detect and localize the tumors. These tests may include:
Talk with your doctor about the best treatment plan for you. Options include:
Surgical removal of the gastrin-secreting tumors may be attempted. This may not be possible if there are multiple tumors or if cancer cells have spread to other parts of the body.
Zollinger-Ellison syndrome may be treated with:
There are no current guidelines to prevent Zollinger-Ellison syndrome. If you have a family history of Zollinger-Ellison syndrome or MEN 1, consider contacting a genetic counselor for screening.
American Gastroenterological Association
National Institute of Diabetes and Digestive and Kidney Diseases
Canadian Association of Gastroenterology
Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National institutes of Health and comparison with 537 cases from the literature, evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine. 2006;85(6):331-364.
Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 2014;19(1):44-50.
Gastrinoma. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T115698/Gastrinoma. Updated May 22, 2017. Accessed February 12, 2018.
Gastrinoma. Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gi-tract/gastrinoma. Updated October 2017. Accessed February 12, 2018.
Ito T, Igarashi H, Jensen RT. Zollinger-Ellison syndrome: recent advances and controversies. Curr Opin Gastroenterol. 2013;29(6):650-661.
Krampitz GW, Norton JA. Current management of the Zollinger-Ellison syndrome. Adv Surg. 2013;47:59-79.
Zollinger-Ellison syndrome. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/zollinger-ellison-syndrome/Pages/facts.aspx. Updated December 2013. Accessed February 12, 2018.
Last reviewed March 2018 by EBSCO Medical Review Board David A. Ostrovsky, MD Last Updated: 3/21/2016