Abstract

BACKGROUND: The Zollinger-Ellison syndrome, implicating a gastrinoma, was first recognized as a disease entity in 1955. At that time, total gastrectomy was the most common treatment approach. Advances in several aspects of the disease have occurred since that time. METHODS: The authors reviewed the changes that have developed since 1955 in the diagnosis, imaging studies, operative and nonoperative management, and follow-up of patients with this disease. RESULTS: The presence of a gastrinoma can be confirmed by a secretin stimulation test. A variable number of patients have hyperparathyroidism as part of the multiple endocrine neoplasm syndrome type 1 (MEN 1). Localization of the primary gastrinoma has been assisted by selective angiography, endoscopic ultrasonography, and the octreotide scan. H2-blockers or omeprazole, sometimes at high doses, usually controls acid secretion. Surgical removal of the primary gastrinoma is performed when feasible, and parathyroidectomy is indicated in those patients with hyperparathyroidism in the MEN 1 syndrome. Follow-up is facilitated by measurement of fasting serum gastrin levels. CONCLUSIONS: Several innovations have improved our capability to diagnose and effectively manage patients with gastrinoma.

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