Long-term survival after pancreaticoduodenectomy for endocrine tumors of the ampulla of vater and minor papilla

OBJECTIVE: Endocrine tumors of the ampullary region are rare, and accurate indications for their management are lacking. We aimed to evaluate the outcome of surgical treatment in this indication. METHOD: We reviewed all patients who submitted to a pancreaticoduodenectomy for ampullary endocrine tumors between 1982 and 2003 in our center. RESULTS: Eight patients, 3 men and 5 women, with a mean age of 47.8 years (range, 37-57 years) were included. Two patients presented with Zollinger-Ellison syndrome, and 1 had neurofibromatosis. Operative mortality was nil. The mean size of the tumors was 17.4 mm (range, 5-40 mm). There were 7 well-differentiated and 1 poorly differentiated endocrine carcinomas. Seven patients had satellite lymph node metastases, and 1 had diffuse liver metastases. Median follow-up was 131 months (range, 17-315 months). At the end of the follow-up period, 5 patients were alive and disease-free; 1 patient was alive with stable liver metastases. Two patients died 17 months and 13 years after surgery, respectively, from metastasis and an unrelated cause. CONCLUSION: This study demonstrates the high frequency of lymph node invasion in these uncommon tumors, even at an early clinical stage. Pancreaticoduodenectomy may result in prolonged survival of patients with well-differentiated tumors.