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.
Background and Methods The role of surgery in patients withthe ZollingerEllison syndrome is controversial. To determinethe efficacy of surgery in patients with this syndrome, we followed151 consecutive patients who underwent laparotomy between 1981and 1998. Of these patients, 123 had sporadic gastrinomas and28 had multiple endocrine neoplasia type 1 with an imaged tumorof at least 3 cm in diameter. Tumor-localization studies andfunctional localization studies were performed routinely. Allpatients underwent surgery according to a similar operativeprotocol, and all patients who had surgery after 1986 underwentduodenotomy.
Results The 151 patients underwent 180 exploratory operations.The mean (±SD) follow-up after the first operation was8±4 years. Gastrinomas were found in 140 of the patients(93 percent), including all of the last 81 patients to undergosurgery. 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 werefree of disease at 10 years, as compared with none of the patientswith multiple endocrine neoplasia type 1. The overall 10-yearsurvival rate was 94 percent.
Conclusions All patients with the ZollingerEllison syndromewho do not have multiple endocrine neoplasia type 1 or metastaticdisease should be offered surgical exploration for possiblecure.
Source Information
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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