Digestive Health

Updated 15 May 2015

Zolinger-Ellison syndrome

A rare syndrome involving tumours in the pancreas and duodenum.



A rare syndrome involving tumours in the pancreas and duodenum which secrete excessive amounts of gastrin, and cause peptic ulcers.

Alternative names


What is this?

An uncommon disorder in which a gastrinoma, a tumour of non-beta islet cells of the duodenum or pancreas ( and occasionally in other unusual places ) , produces excessive quantities of the hormone gastrin, causing the stomach to secrete excessive amounts of acid, leading to peptic ulcers.

What causes this?

Why people with ZE develop the tumours in the first place is unknown, though around one-quarter of them have a genetic anomaly which is related to other growths, in the form of multiple endocrine neoplasia, MEN1. There may be an individual gastrinoma, or a number of smaller tumours. The gastrin causes greatly increased production of stomach acid, both by increasing the number of cells which produce acid, and by stimulating each of these cells to produce more acid than usual.

What are its symptoms?

Typical symptoms of peptic ulcers, such as a burning, gnawing, pain in the belly, and the symptoms don't respond to ordinary remedies such as antacids. Also, nausea and vomiting, diarrhoea, weakness, weight-loss and bleeding from the ulcers. This may occur at any age, but usually appears around the age of 50.

How is it diagnosed?

Tests can measure the amount of gastrin being produced, and the amount of stomach acid being generated. Endoscopy, using a lighted flexible tube to examine the lining of the stomach and duodenum, is also usually helpful. The ulcers produced in this syndrome are more numerous, and often in unusual parts of the stomach. Imaging (scans) may help to locate the tumour(s).

How is it treated?

The ulcers caused by the Zollinger-Ellison Syndrome respond less well to ulcer treatments than do ordinary ulcers. The aim is to reduce the production of stomach acid, using such drugs as the proton pump inhibitors such as omeprazole, and H-2 blockers such as Cimetidine and Ranitidine, though these are not as effective as usual. Surgery may be used to remove the ulcers and the tumours giving rise to them. Chemotherapy can reduce the tumours.

What is the prognosis?

There is a good chance of bringing the condition under control, though there needs to be continuing monitoring, as it can recur. A proportion of the single gastrinomas (some estimate as many as a half or more ) are malignant, and spread to the liver and to nearby lymph nodes.

When to call your doctor .

If you have been experiencing severe and persistent ulcer-type pains, especially if these are accompanied by nausea, vomiting and diarrhoea, consult your doctor. And don't take over-the-counter, non-prescription antacids for long periods of time without consulting the doctor.

How can it be prevented?

It is not preventable, but early recognition and treatment greatly improves the results.


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Digestive Health Expert

Dr. Estelle Wilken is a Senior Specialist in Internal Medicine and Gastroenterology at Tygerberg Hospital. She obtained her MBChB in 1976, her MMed (Int) in 1991 and her gastroenterology registration in 1995.

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