Surgery to cure the Zollinger-Ellison syndrome

Background and Methods The role of surgery in patients with the Zollinger-E llison syndrome is controversial. To determine the efficacy of surgery in p atients with this syndrome, we followed 151 consecutive patients who underw ent laparotomy between 1981 and 1998. Of these patients, 123 had sporadic g astrinomas and 28 had multiple endocrine neoplasia type 1 with an imaged tu mor of at least 3 cm in diameter. Tumor-localization studies and functional localization studies were performed routinely. All patients underwent surg ery according to a similar operative protocol, and all patients who had sur gery 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 pat ients to undergo surgery. The tumors were located in the duodenum in 74 pat ients (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 un known in 24 patients (16 percent). Among the patients with sporadic gastrin omas, 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 hav e multiple endocrine neoplasia type 1 or metastatic disease should be offer ed surgical exploration for possible cure. (N Engl J Med 1999;341:635-44.) (C) 1999, Massachusetts Medical Society.