Zollinger Ellinson syndrome - Disorders of the stomach

Zollinger–Ellinson syndrome

Definition

Pathological secretion of gastrin resulting in hypersecretion of acid.

Aetiology/pathophysiology

The condition is usually caused by a gastrinoma in the G cells of the pancreatic islets it occurs most commonly in males between 20–50 years of age. 60–90% of gastrinomas are malignant often with metastases at diagnosis.

Clinical features

Patients present with epigastric burning pain and with complications of peptic ulcer disease. The excess acid causes inactivation of duodenal/jejunal lipases and hence steatorrhoea also occurs.

Investigations

A fasting serum gastrin level is taken (>150 ng/L is suggestive, >500 ng/L strongly suggestive). The patient should not be taking a proton pump inhibitor as these increase gastrin levels. Tumour location is attempted with isotope scanning and CT of the abdomen.

Management

Resection of the gastrinoma should be attempted but problems with locating the tumour, which is often multifocal, makes surgery difficult. High-dose proton pump inhibitors are also used. Other treatment options include octreotide, interferon α, chemotherapy and hepatic artery embolisation.

Prognosis

In inoperable tumours 60% of patients survive 5 years and 40% survive 10 years.