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Surgery to cure the Zollinger-Ellison syndrome
J A Norton1, D L Fraker, H R Alexander
1Department of Surgery, University of California, San Francisco, and the San Francisco Veterans Affairs Medical Center, USA.
The New England Journal of Medicine
|August 26, 1999
Summary
Surgical exploration is recommended for Zollinger-Ellison syndrome patients without MEN1 or metastatic disease. This study found a 94% overall survival rate, with 34% of sporadic gastrinoma patients disease-free at 10 years.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Endocrinology
Background:
- The efficacy of surgical intervention for Zollinger-Ellison syndrome remains debated.
- Zollinger-Ellison syndrome is characterized by gastrin-producing tumors (gastrinomas).
Purpose of the Study:
- To evaluate the effectiveness of surgical exploration in managing Zollinger-Ellison syndrome.
- To assess long-term outcomes and survival rates following surgical treatment.
Main Methods:
- A cohort of 151 patients with Zollinger-Ellison syndrome underwent laparotomy between 1981 and 1998.
- Patients included those with sporadic gastrinomas and multiple endocrine neoplasia type 1 (MEN1).
- Tumor localization studies and surgical protocols were consistently applied.
Main Results:
- Gastrinomas were identified in 93% of patients.
- Tumor locations included the duodenum (49%), pancreas (24%), and lymph nodes (11%).
- Ten-year disease-free rates were 34% for sporadic gastrinomas versus 0% for MEN1; overall 10-year survival was 94%.
Conclusions:
- Surgical exploration should be offered to all Zollinger-Ellison syndrome patients without MEN1 or metastatic disease.
- Surgery offers a potential cure and improves survival outcomes.
- Early and accurate tumor localization is crucial for successful surgical management.