title: Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.
abstract: OBJECTIVE
  To describe the biological behavior and surgical management of ampullary neuroendocrine tumors in 7 patients.
  
  
  METHODS
  Case series and literature review.
  
  
  METHODS
  University hospital.
  
  
  METHODS
  Seven patients with ampullary neuroendocrine tumors.
  
  
  METHODS
  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.
authors.affiliation: Department of Surgery, University of California, San Francisco, 4 Koret Way, LR-101, Box 0475, San Francisco, CA 94143-0475, USA. jonathan.carter@ucsfmedctr.org
authors.forename: Jonathan T
authors.initials: JT
authors.lastname: Carter
authors.forename: James P
authors.initials: JP
authors.lastname: Grenert
authors.forename: Laura
authors.initials: L
authors.lastname: Rubenstein
authors.forename: Lygia
authors.initials: L
authors.lastname: Stewart
authors.forename: Lawrence W
authors.initials: LW
authors.lastname: Way
mesh_terms: D014670:Ampulla of Vater; D001706:Biopsy; D003138:Common Bile Duct Neoplasms / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000601:surgery*; D016577:Pancreaticoduodenectomy