Management and prognosis of the Zollinger-Ellison syndrome (gastrinoma)

INTRODUCTION

Patients with Zollinger-Ellison syndrome (ZES) have gastrin-secreting tumors and the associated clinical consequences. This disorder can occur sporadically, or as a manifestation of multiple endocrine neoplasia type 1 (MEN 1). Medical therapy is the current standard of care for most patients with ZES as part of the MEN 1 syndrome. By contrast, many patients with sporadic ZES are candidates for surgical therapy. (See "Clinical manifestations and diagnosis of Zollinger-Ellison syndrome (gastrinoma)" and "Multiple endocrine neoplasia type 1: Treatment" and 'Surgery' below.)

Prior to the development of effective acid suppression therapy, the major morbidity and mortality of ZES were related to complications of fulminant peptic ulcer disease; total gastrectomy was the only effective measure to protect patients from these problems [1].

The development of H2 antagonists and the more powerful proton pump inhibitors has resulted in a significant decrease in morbidity and mortality from ulcer disease and has obviated the need for gastrectomy [2]. Of 212 patients with ZES studied prospectively for a mean of 13.8 years, a ZES-related cause of death could be identified in only half of the 31 percent who died. All of the ZES-related deaths were due to tumor spread; none were due to hypersecretory complications [3].

This topic review will discuss the two current goals of therapy in ZES [4]:

Control of the complications resulting from autonomous release of gastrin

                

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Literature review current through: Jun 2014. | This topic last updated: Oct 10, 2013.
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References
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