Definition of Zollinger-Ellison syndrome

Zollinger-Ellison syndrome: A rare disorder caused by a tumor called a gastrinoma, most often occurring in the pancreas. The tumor secretes the hormone gastrin, which causes increased production of gastric acid leading to severe recurrent ulcers of the esophagus, stomach, and the upper portions of the small intestine (the duodenum and jejunum).

Gastrinomas resulting in the Zollinger-Ellison's syndrome are not limited to the pancreas but may also occur in the stomach, duodenum, spleen and lymph nodes.

The treatment of the Zollinger-Ellison's syndrome includes the use of H2 antagonists (for example cimetidine [brand name: Tagamet] and ranitidine [Zantac]) and the proton pump inhibitors (for example, lansoprazole [Prevacid] and omeprazole [Prilosec]). The H2 antagonists block the action of histamine on stomach cells, thus reducing stomach acid production. The proton pump inhibitors also block the production of acid by the stomach cells. Proton pump inhibitors are more effective than H2 antagonists in suppressing acid and have to be used in very high doses. Surgical removal of the tumor is curative in about 25% of cases.

The syndrome is named for two American surgeons Robert M. Zollinger (1903-1992) and Edwin H. Ellison (1918-1970).