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Original Article
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Volume 341:635-644 August 26, 1999 Number 9
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Surgery to Cure the Zollinger–Ellison Syndrome
Jeffrey A. Norton, M.D., Douglas L. Fraker, M.D., H. Richard Alexander, M.D., David J. Venzon, Ph.D., John L. Doppman, M.D., Jose Serrano, M.D., Ph.D., Stephan U. Goebel, M.D., Paolo L. Peghini, M.D., Praveen K. Roy, M.D., Fathia Gibril, M.D., and Robert T. Jensen, M.D.

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ABSTRACT

Background and Methods The role of surgery in patients with the Zollinger–Ellison syndrome is controversial. To determine the efficacy of surgery in patients with this syndrome, we followed 151 consecutive patients who underwent laparotomy between 1981 and 1998. Of these patients, 123 had sporadic gastrinomas and 28 had multiple endocrine neoplasia type 1 with an imaged tumor of at least 3 cm in diameter. Tumor-localization studies and functional localization studies were performed routinely. All patients underwent surgery according to a similar operative protocol, and all patients who had surgery after 1986 underwent duodenotomy.

Results The 151 patients underwent 180 exploratory operations. The mean (±SD) follow-up after the first operation was 8±4 years. Gastrinomas were found in 140 of the patients (93 percent), including all of the last 81 patients to undergo surgery. The tumors were located in the duodenum in 74 patients (49 percent) and in the pancreas in 36 patients (24 percent); however, primary tumors were found in lymph nodes in 17 patients (11 percent) and in another location in 13 patients (9 percent). The primary location was unknown in 24 patients (16 percent). Among the patients with sporadic gastrinomas, 34 percent were free of disease at 10 years, as compared with none of the patients with multiple endocrine neoplasia type 1. The overall 10-year survival rate was 94 percent.

Conclusions All patients with the Zollinger–Ellison syndrome who do not have multiple endocrine neoplasia type 1 or metastatic disease should be offered surgical exploration for possible cure.


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From the Department of Surgery, University of California, San Francisco, and the San Francisco Veterans Affairs Medical Center, San Francisco (J.A.N.); the Surgical Metabolism Section, Surgery Branch, National Cancer Institute, Bethesda, Md. (D.L.F., H.R.A.); the Biostatistics and Data Management Section, National Cancer Institute, Bethesda, Md. (D.J.V.); the Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Md. (J.L.D.); and the Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Md. (J.S., S.U.G., P.L.P., P.K.R., F.G., R.T.J.).

Address reprint requests to Dr. Jensen at NIH/NIDDK/DDB, Bldg. 10, Rm. 9C-103, 10 Center Dr., MSC 1804, Bethesda, MD 20892-1804, or at robertj{at}bdg10.niddk.nih.gov.

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Surgery to Cure the Zollinger–Ellison Syndrome
Hirschowitz B. I., Norton J. A., Gibril F., Jensen R. T.
Extract | Full Text  
N Engl J Med 1999; 341:2096-2097, Dec 30, 1999. Correspondence

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