Duodenopancreatic resections in patients with multiple endocrine neoplasia type 1

Abstract

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 tumor with limited complications, preserving pancreatic function.

Summary background data: MEN 1 is an autosomal dominant familial neoplasia syndrome characterized by the development of NETs of the duodenum and pancreas. Some tumors are clinically insignificant or follow a benign course, although a subset pursues a malignant, lethal natural history; the risk of surgical management must be appropriate to the disease course.

Methods: The clinical, biochemical, genetic, and pathologic data were retrospectively reviewed for 21 consecutive MEN 1 patients undergoing pancreatic resection for NETs between 1993 and 1999 at one institution. Age at operation, presenting symptoms, results of preoperative and intraoperative localization studies, major and minor complications, and pathology, including metastases, were analyzed.

Results: The surgical approach was selected based on the location and size of the tumors. Five patients required pancreaticoduodenectomy, 11 patients underwent non-Whipple pancreatic resections, and 5 underwent simple enucleation of benign NETs. The incidence of regional lymph node metastases was 33%.

Conclusions: Major pancreatic procedures can be performed safely in most patients with MEN 1 and NETs. Because NETs are the most common MEN 1-related cause of death in the authors' kindreds, an aggressive surgical approach, including early intervention before malignant spread and major pancreatic resection where indicated, appears justified.

Figure 1. Distribution of primary duodenal and pancreatic neuroendocrine tumors in 21 patients with multiple endocrine neoplasia type 1. Two gastrinomas were located in the duodenal wall. The intrapancreatic tumors demonstrated an even distribution according to the approximate volume of parenchyma in each pancreatic region. Maximum diameter of largest tumor: N = 39; range 0.1–8.0 cm; mean 1.83 cm.

Figure 2. Scatter plot depicting the relation between the maximum diameter of the largest primary tumor and the presence of regional lymph node (LN) or distant metastases. There was significant overlap between all groups. The relation between tumor size and the presence of metastases (group I = no metastases, group II = LN metastases plus distant metastases) was not significant (P = .1135, Mann-Whitney test).