ZOLLINGER-ELLISON SYNDROME

The Zollinger-Ellison syndrome is an uncommon but not rare condition characterized by neuroendocrine tumors that secrete the gastrointestinal regulatory peptide gastrin. Although originally thought to occur exclusively within pancreatic islets, gastrinomas are also commonly located in the duodenal wall, as well as in extrapancreatic, extraintestinal sites. Early reports emphasized morbidity and mortality associated with the hypersecretion of gastric acid caused by exceedingly elevated levels of circulating gastrin. With the ability to control the hyperchlorhydria, initially by total gastrectomy and more recently by the use of potent antisecretory agents, however, patients are now succumbing more often to the malignant behavior of these generally slow-growing tumors. Therefore, surgical extirpation of these tumors has become the treatment most commonly recommended. With the careful use of techniques aimed at tumor localization, including computed tomography, selective angiography, and thorough exploratory laparotomy, it is estimated that at least 20% of all patients with the Zollinger-Ellison syndrome can be cured.