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

Objective: To evaluate the outcome of an aggressive surgical approach for duodenopancreatic neuroendocrine tumors (PETs) associated with multiple endocrine neoplasia type 1 (MEN1). Summary Background Data: The management of PETs is still controversial in the setting of the autosomal dominant inherited MEN1-syndrome. Methods: MEN1 patients that had either biochemical evidence of functioning PETs or visualized non-functioning PETs larger than 1 cm in size on imaging were operated on. Since 1997 patients were followed annually by biochemical testing and imaging studies. Results: Twenty six genetically confirmed MEN1 patients underwent duodenopancreatic resection for functioning (n=17) or nonfunctioning (n=9) PETs. Ten (38%) patients had malignant PETs as characterized by the presence of lymph node (10 patients) and/or distant metastases (2 patients). The surgical approach was selected based on the type, location and size of PETs. Four ZES patients required pylorus preserving pancreaticoduodenectomy (PPPD) as initial or redo procedure, 20 patients underwent other duodenopancreatic resections and 2 patients had single enucleations of PETs. After median 83 (range 5-241) months 24 patients were alive and 2 patients were deceased of an unrelated cause. All patients with insulinoma or vipoma and 7 of 11 patients with ZES were biochemically cured, 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 detectable metastases on imaging. Conclusion: Early and aggressive surgery of PETs in MEN1 patients prevents the development of liver metastases which are the most life-threatening determinant. PPPD might be the procedure of choice for MEN1-ZES, which has to be proven in large scale studies.