Zollinger-Ellison syndrome: An analysis of twenty-five cases☆☆☆

Abstract Multiple endocrine adenomatosis was identified in 48 per cent of 25 patients with the Zollinger-Ellison syndrome. This figure is twice that in Ellison's series and is probably closer to the true incidence. Eleven patients had hyperparathyroidism, five had adrenal hyperplasia or adenomas, four had pituitary tumors, three had thyroid tumors, one had carcinoid tumors, and two had hyperinsulinism. It should be emphasized that a patient with hyperparathyroidism and peptic ulcer disease is likely to have ulcerogenic pancreatic tumors. All patients with the Zollinger-Ellison syndrome should be investigated for hyperparathyroidism. Diagnosis is often difficult with presently available technics. Positive information in the form of a high basal to maximal acid output ratio, bioassay positive for gastrin, or demonstration of an islet cell tumor is quite reliable. Negative information is less helpful in attempting to exclude the diagnosis. Total gastrectomy has been very successful as treatment for this disease. Since it is curative and is associated with little nutritional morbidity in these patients, total gastrectomy is the only recommended operation except in patients with primary ectopic tumors; these patients are successfully treated by tumor excision.