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(Z-E Syndrome; Gastrinoma)
by Dianne Scheinberg, MS, RD, LDN
Zollinger-Ellison syndrome is a rare disorder that arises from tumors and ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). These tumors, called gastrinomas, produce a large amount of gastrin. Gastrin is a hormone that causes the stomach to produce acid. With too much gastrin, excess acid is produced, causing ulcers in the stomach or small intestine.
Gastrinomas occur as single tumors or small multiple tumors. Not only can these tumors lead to ulcers, they can also be cancerous (up to 66% malignant) and spread to the nearby lymph nodes or liver. This happens in about one-third to one-half of the cases of Zollinger-Ellison syndrome.
About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). Patients with MEN 1 have additional endocrine tumors in the brain and neck.
The cause of Zollinger-Ellison syndrome is unclear. It is very rare; fewer than three out of a million people have the syndrome.
Risk Factors TOP
Factors that may increase the chance of MEN 1 include:
Over 90% of people with Zollinger-Ellison syndrome have symptoms typical of a stomach ulcer.
If you experience any of these symptoms, do not assume it is due to Zollinger-Ellison syndrome. These symptoms may be caused by other, less serious health conditions:
Your doctor will ask about your symptoms and medical history, and perform a physical exam. He or she may also refer you to a gastroenterologist, a specialist who deals with gastrointestinal disorders.
Tests may include the following:
Talk with your doctor about the best treatment plan for you. Treatment options include:
Surgical Removal of Tumor
If there is only one tumor and it is not cancerous, a surgical removal may be attempted.
Medications for Ulcers
There are no know prevention steps since the cause is unclear.
American Gastroenterological Association
National Digestive Diseases Information Clearinghouse
The Canadian Association of Gastroenterology
Berna MJ, Hoffmann KM, Long SH, et al. 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;85:331-64.
Campana D, Piscitelli L, Mazzotta E, et al. Zollinger-Ellison syndrome: diagnosis and therapy. Minerva Med. 2005;96:167-206.
DynaMed Editorial Team. Zollinger-Ellison syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated October 18, 2010. Accessed November 11, 2010.
Mayo Clinic. Zollinger-Ellison syndrome. Mayo Clinic website. Available at: http://www.mayocli... . Updated July 24, 2010. Accessed November 11, 2010.
Norton JA, Fraker DL, Alexander HR, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med . 1999;341:635-644.
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Tomassetti P, Salomone T, Migliori M, et al. Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. Drgus Aging. 2003;20:1019-1034.
Last reviewed March 2013 by Daus Mahnke, MD
Last Updated: 03/15/2013