Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.

OBJECTIVES To describe the biological behavior and surgical management of ampullary neuroendocrine tumors in 7 patients. DESIGN Case series and literature review. SETTING University hospital. PATIENTS Seven patients with ampullary neuroendocrine tumors. MAIN OUTCOME MEASURES Clinical presentation, pathologic findings, and survival. RESULTS The patients presented with jaundice (3 patients), anemia (1 patient), gastric outlet obstruction (1 patient), or incidental discovery (2 patients). No patients had neurofibromatosis. Preoperative biopsy was diagnostic in 5 of 6 patients. All of the tumors expressed chromogranin and synaptophysin. Even when the tumor expressed gastrin, vasoactive intestinal peptide, or somatostatin, no patient had a hypersecretion syndrome. Five patients were treated by pancreaticoduodenectomy, 4 for low-grade neuroendocrine tumors and 1 for high-grade neuroendocrine carcinoma. The lesions measured 1.0 to 3.5 cm in diameter. Computed tomographic scans failed to detect nodal metastases that were present in 4 patients. One patient with a high-grade malignant neoplasm died after 15 months. The rest were disease-free after 19 to 48 months. Two patients had transduodenal local resections, one for a 1.1-cm paraganglioma (disease-free, 11 years) and the other for a 0.6-cm carcinoid tumor (disease-free, 7 months). CONCLUSIONS This is one of the largest series of neuroendocrine tumors of the ampulla. Preoperative biopsy was accurate, but computed tomographic scans were insensitive in detecting nodal metastases. Unlike duodenal carcinoid tumors, hypersecretion syndromes were absent and small tumor size did not preclude locoregional metastases. Tumor grade predicted survival. We recommend pancreaticoduodenectomy for this disease, with local resection reserved for mobile, superficial lesions.