Surgical treatment of duodenopancreatic neuroendocrine tumors (PETs) associated with multiple neoplasia type 1 (MEN1).

Objective: To evaluate the outcome of an aggressive surgical approachfor duodenopancreatic neuroendocrine tumors (PETs) associatedwith multiple endocrine neoplasia type 1 (MEN1).Summary Background Data: The management of PETs is stillcontroversial in the setting of the autosomal dominant inheritedMEN1-syndrome.Methods: MEN1 patients that had either biochemical evidence offunctioning PETs or visualized non-functioning PETs larger than 1cm in size on imaging were operated on. Since 1997 patients werefollowed annually by biochemical testing and imaging studies.Results: Twenty six genetically confirmed MEN1 patients underwentduodenopancreatic resection for functioning (n=17) or nonfunctioning(n=9) PETs. Ten (38%) patients had malignant PETs ascharacterized by the presence of lymph node (10 patients) and/ordistant metastases (2 patients). The surgical approach was selectedbased on the type, location and size of PETs. Four ZES patientsrequired pylorus preserving pancreaticoduodenectomy (PPPD) asinitial or redo procedure, 20 patients underwent other duodenopancreaticresections and 2 patients had single enucleations of PETs.After median 83 (range 5-241) months 24 patients were alive and 2patients were deceased of an unrelated cause. All patients withinsulinoma or vipoma and 7 of 11 patients with ZES were biochemicallycured, including the ZES patients who underwent PPPD.However, 19 of 26 (73%) patients developed new small PETs(<1cm) in the pancreatic remnant, but no patient had yet detectablemetastases on imaging.Conclusion: Early and aggressive surgery of PETs in MEN1 patientsprevents the development of liver metastases which are themost life-threatening determinant. PPPD might be the procedure ofchoice for MEN1-ZES, which has to be proven in large scale studies.