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Causes
By Mayo Clinic staff
The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.
Your pancreas is located behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones, including gastrin, a hormone that controls stomach acid production. The duodenum, the upper part of the small intestine, begins at the lower end of your stomach. It's in the duodenum, where digestive juices from the pancreas, liver and gallbladder mix, that digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete very large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.
In addition to causing excess acid production, the tumors may be cancerous (malignant). The tumors themselves grow slowly, but the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
Association with MEN I
Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type I (MEN I). People with MEN I have multiple tumors in the endocrine system in addition to pancreatic tumors. They also have tumors in the parathyroid glands and may have tumors in their pituitary glands. About 25 percent of people who have gastrinomas have them as part of MEN I.
- Zollinger-Ellison syndrome. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/zollinger. Accessed May 5, 2010.
- How is cancer of the pancreas diagnosed? American Cancer Society. http://www.cancer.org/docroot/CRI/content/CRI_2_4_3X_How_is_pancreatic_cancer_diagnosed_34.asp?sitearea=. Accessed May 5, 2010.
- Goldfinger SE. Clinical manifestations and diagnosis of Zollinger-Ellison syndrome (gastrinoma). http://www.uptodate.com/home/index.html. Accessed May 8, 2010.
- Goldfinger SE. Management and prognosis of Zollinger-Ellison syndrome (gastrinoma). http://www.uptodate.com/home/index.html. Accessed May 8, 2010.
- Morrow EH, et al. Surgical management of Zollinger-Ellison syndrome; State of the art. Surgical Clinics of North America. 2009;89:1091.
- Wilcox CM, et al. Treatment strategies for Zollinger-Ellison syndrome. Expert Opinion on Pharmacotherapy. 2009;10:1145.
- FDA Drug Safety Communication: Possible increased risk of fractures of the hip, wrist, and spine with the use of proton pump inhibitors. http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformationforPatientsandProviders/ucm213206.htm. Accessed May 26, 2010.
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