Duodenopancreatic resections in patients with multiple endocrine neoplasiatype 1

Objective To review the authors' 7-year experience with a surgical approach for pancreatic and duodenal neuroendocrine tumors (NETs) in patients with multiple endocrine neoplasia type 1 (MEN 1) designed to remove all gross tu mor with limited complications, preserving pancreatic function. Summary Background Data MEN 1 is an autosomal dominant familial neoplasia s yndrome characterized by the development of NETs of the duodenum and pancre as. Some tumors are clinically insignificant or follow a benign course, alt hough a subset pursues a malignant, lethal natural history, the risk of sur gical management must be appropriate to the disease course. Methods The clinical, biochemical, genetic, and pathologic data were retros pectively reviewed for 21 consecutive MEN 1 patients undergoing pancreatic resection for NETs between 1993 and 1999 at one institution. Age at operati on, presenting symptoms, results of preoperative and intraoperative localiz ation studies, major and minor complications, and pathology, including meta stases, were analyzed. Results The surgical approach was selected based on the location and size o f the tumors. Five patients required pancreaticoduodenectomy, 11 patients u nderwent non-Whipple pancreatic resections, and 5 underwent simple enucleat ion of benign NETs. The incidence of regional lymph node metastases was 33% . Conclusions Major pancreatic procedures can be performed safely in most pat ients with MEN 1 and NETs. Because NETs are the most common MEN I-related c ause of death in the authors' kindreds, an aggressive surgical approach, in cluding early intervention before malignant spread and major pancreatic res ection where indicated, appears justified.