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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 or duodenum.
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 insulin and glucagon, both of which regulate blood sugar levels, as well as the stomach hormone gastrin, which controls production of stomach acid. The duodenum, the upper part of the small intestine, begins at the lower end of your stomach. In the duodenum, digestive juices from the pancreas, liver and gallbladder mix, and 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 cause 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 1
Zollinger-Ellison syndrome may be associated with another disease called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 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 1.
