Surgery for a gastroenteropancreatic neuroendocrine tumor (GEPNET) in multiple endocrine neoplasia type 1

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited endocrine tumor syndrome characterized by tumor development in various endocrine organs such as the parathyroid, endocrine pancreas, anterior pituitary and adrenal cortex. The first extensive database for Asian patients with MEN1 was established by the MEN Consortium of Japan. Although the clinical features of Japanese patients with MEN1 are similar to those from Western countries, there are several characteristic differences between them. In particular, gastroenteropancreatic neuroendocrine tumor (GEPNET) is seen in approximately 60% of patients with MEN1 in Japan. Although its development is the strongest prognostic factor in patients with MEN1, the characteristics of MEN1-associated GEPNET still remain unclear. This is especially true for the differences in clinical features of GEPNET with and without MEN1. Improved long-term survival is obtained by curative surgery for patients with MEN1-associated GEPNET. The current surgical indications are expanding even in patients with hepatic metastases because of the improved surgical outcome. This article reviews the clinical characteristics in these patients with a particular focus on surgery, diagnosis, surgical indications, surgical method, and surgical outcome.