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The Role of Surgery in the Zollinger-Ellison Syndrome

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TLDR
Treatment of the hypersecretory problems of the Zollinger-Ellison syndrome by total gastrectomy is safe and dependable, and results compare well with those of long-term medical management, whose success is dependent upon serial favorable responses to a lifetime of repeated challenges.
Abstract
When the Zollinger-Ellison syndrome was first identified, total gastrectomy was proposed as the most effective treatment for the secretory manifestations of the syndrome. Recently, however, great enthusiasm has developed for medical treatment by means of H2-receptor antagonists. The authors have cared for 27 patients with the Zollinger-Ellison syndrome at The University of Texas Medical Branch in the past 12 years and have been pleased with the results of total gastrectomy, which was performed in 23 of the 27 patients (one patient refused operation and three patients had lesser gastric operations). Twenty-three patients underwent total gastrectomy with Roux-en-Y esophagojejunostomy. There were no operative deaths. Primary tumors were found in 17 patients, seven of whom also had metastatic tumors. No tumors were found in nine patients. Nine patients are dead; the actuarial survival rate for all patients was 75% at 5 years and 52% at 10 years. Eleven of the 27 patients had the multiple endocrine neoplasia I syndrome. Of the 18 survivors, only three have normal serum gastrin levels, and all three had extrapancreatic gastrinomas, one in peripancreatic lymph nodes, one in the liver, and one in a cystic tumor attached to the stomach. Nutritional results were good to excellent, with a mean postoperative weight loss of 14.7% (mean follow-up period was 45 months). The authors conclude that treatment of the hypersecretory problems of the Zollinger-Ellison syndrome by total gastrectomy is safe and dependable. Results compare well with those of long-term medical management, whose success is dependent upon serial favorable responses to a lifetime of repeated challenges.

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Journal ArticleDOI

ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes.

TL;DR: The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rarefunctional p- NETs are considered together as a group called rare functional rFTs (RFTs).
Journal ArticleDOI

Determinants of Metastatic Rate and Survival in Patients With Zollinger-Ellison Syndrome: A Prospective Long-term Study

TL;DR: Survival was primarily determined by the presence of liver metastases, and the frequency of Liver metastases depends on the size and location of the primary tumor and on the Presence of MEN-1 at the initial presentation.
Journal ArticleDOI

Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome

TL;DR: Gastrinoma growth is now the main single determinant of long-term survival in ZES, with one half of patients dying a gastrinoma-related death and none an acid- related death.
Journal ArticleDOI

The gastrinoma triangle: Operative implications

TL;DR: An aggressive approach towards curative tumor excision is now advocated for all gastrinoma patients who are suitable operative risks and have no evidence preoperatively of liver metastases or the multiple endocrine neoplasm-type I syndrome.
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