What is Zollinger-Ellison syndrome?

Zollinger-Ellison Syndrome (ZES) is a condition where increased production and release of stomach acid is associated with the formation of ulcers. This is an uncommon cause of stomach and duodenal ulcers (peptic ulcers) as most are actually caused by bacteria called Helicobacter pylori (H. pylori). Most peptic ulcer disease caused by H. pylori can be treated effectively with acid reducing medications and antibiotics. ZES can be distinguished from the common cause of peptic ulcer disease in most cases, namely the presence of multiple or atypical ulcers. Atypical or multiple ulcers strongly suggest the diagnosis of ZES and therefore a gastrinoma, however in 18% to 25% of patients no ulcers are present at the time of diagnosis. 

Zollinger-Ellison syndrome is a rare condition in which a tumor called a gastrinoma forms in the pancreas or small intestine. The tumor releases a hormone called gastrin that causes production of excess stomach acid.

Almost all people who have Zollinger-Ellison syndrome get an ulcer at some point, usually in the small intestine (duodenal ulcer). Ulcers produced by this disease are more difficult to cure than ulcers from other causes.

In about half of the people with Zollinger-Ellison syndrome, the tumors (gastrinomas) are cancerous and can spread to the lymph nodes and liver.

The main treatment for Zollinger-Ellison syndrome is removal of the tumors and taking acid-suppressing medicine called proton pump inhibitors.