Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1. Surgery or surveillance?

Background, The management of pancreaticoduodenal endocrine tumors (PETs) r emains controversial in multiple endocrine neoplasia type 1 (MEN 1). Methods. Twenty-one patients with MEN 1 and PETs were analyzed for outcome of surgery and surveillance with special regard to the genotype based on ME N1 gene mutation analysis. Results. Nine patients had gestrinomas, 5 had nonfunctioning tumors, 4 had insulinomas, 2 had insulinomas and gastrinomas, and 1 had a VIPoma. Seven p atients (33%) had malignant tumors. Sixteen patients (76%) were initially t reated by pancreatic resections or tumor enucleations or both. Six patients underwent reoperations for recurrences or lymph node metastases or both. F ifteen of the 16 operated patients are alive, and 12 have no evidence of di sease after a median follow-up of 78 months (range, 1-198 months). Five pat ients with gastrinomas or nonfunctioning tumors, but no symptoms, underwent surveillance; 1 of them developed lymph node metastases. Patients with tru ncating mutations in the N- or C-terminal region (exons 2, 9, or 10) of the MEN1 gene had a significantly higher rate of malignant tumors (55% vs 10%; P <.05) than patients with other mutations. Conclusions. An aggressive surgical approach is justified for PETs in patie nts with MEN1. However, MEN1. gene mutations in exons 3 to 8 seem to be ass ociated with mild behavior of PETs, possibly allowing surveillance in asymp tomatic patients.