Management and prognosis of the Zollinger-Ellison syndrome (gastrinoma)
- Stephen E Goldfinger, MD
Stephen E Goldfinger, MD
- Professor of Medicine
- Harvard Medical School
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 "Zollinger-Ellison syndrome (gastrinoma): Clinical manifestations and diagnosis" 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 .
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 . 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 .
This topic review will discuss the two current goals of therapy in ZES :
●Control of the complications resulting from autonomous release of gastrin
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- MEDICAL MANAGEMENT
- Proton pump inhibitors
- Reoperation for recurrence
- RADIATION THERAPY FOR NONSURGICAL CANDIDATES
- THERAPY OF METASTATIC DISEASE
- Somatostatin analogs
- Liver-directed therapy
- - Resection
- - Hepatic artery embolization
- - RFA and cryoablation
- - Liver transplantation
- Chemotherapy and novel treatment approaches
- POSTTREATMENT SURVEILLANCE
- SUMMARY AND RECOMMENDATIONS