Zollinger Ellison syndrome

In this rare disorder a gastrin secreting neuroendocrine tumour of the pancreas ('gastrinoma') is present with recurrent duodenal ulceration and diarrhoea. It accounts for only about 0.1% of all cases of duodenal ulceration (Misiewicz and Punder 2000). The syndrome occurs in either sex at any age, although it is most common between 30 and 50 years. Patients with Zollinger-Ellison syndrome present with pain as the predominant symptom from peptic ulcer disease; they also have diarrhoea and steatorrhoea.

Peptic ulcers are often multiple and severe and there is a poor response to standard ulcer medical therapy. The history is usually short; bleeding and perforations are common.

Pathophysiology of Zollinger-Ellison syndrome

Tumours mostly occur in the pancreatic head or tail (Misiewicz and Punder 2000). The gastrinoma secretes large amounts of gastrin, which stimulates the parietal cells of the stomach to secrete acid to their maximal capacity and increases the parietal cell mass three to sixfold. The acid output may be so great that it reaches the upper small intestine, reducing the luminal pH to 2 or less. Pancreatic lipase is inactivated and bile acids are precipitated.

An increased serum gastrin is the most specific and reliable test for Zollinger-Ellinson syndrome. Gastrin levels are grossly elevated (ten to one thousandfold). Tumour localisation is often attempted using a CT scan.

Management of Zollinger-Ellison syndrome

Approximately 30% of small and single tumours can be localised and resected but many tumours are multifocal. Some patients present with metastatic disease and surgery is inappropriate. Advances in medical treatment have made total gastrectomy unnecessary, and in the majority of patients continuous therapy with proton pump inhibitors heals ulcers and alleviates diarrhoea. Larger doses (60-80 mg daily) than those used to treat duodenal ulcer are required.

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