The Role of Surgery in the Zollinger-Ellison Syndrome
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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.read more
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Journal ArticleDOI
Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)
Rajesh V. Thakker,Paul J. Newey,Gerard V Walls,John P. Bilezikian,Henning Dralle,Peter R. Ebeling,Shlomo Melmed,Akihiro Sakurai,Francesco Tonelli,Maria Luisa Brandi +9 more
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ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes.
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H. Christian Weber,David Venzon,Jaw-Town Lin,Vitaly A. Fishbein,Murray Orbuch,Doris B. Strader,Fathia Gibril,David C. Metz,Douglas L. Fraker,Jeffrey A. Norton,Robert T. Jensen +10 more
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Fang Yu,David Venzon,Jose Serrano,Stephan U. Goebel,John L. Doppman,Fathia Gibril,Robert T. Jensen +6 more
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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.