Background: Carcinoid of the ampulla of Vater is a rare lesion that may produce obstructive jaundice. Analysis of nodal involvement as a function of primary tumor sizes indicated that metastases occur with tumors smaller than 2.0 cm. The case of a 36-year-old woman is presented and the literature reviewed in an attempt to define the treatment of choice.
Methods: The English literature was reviewed, and 27 cases including the current one were identified. All cases were traced to the original case report. Clinical outcome data were tabulated.
Results: The mean age of the sample group was 48.5 years, with 52% men and 48% women. Jaundice occurred in 60% of patients. Ampullary carcinoid developed in nine patients in association with von Recklinghausen disease. Treatment included pancreaticoduodenectomy, local excision, and biliary-enteric and endoscopic stenting. Seven patients had positive nodes. Four had metastatic disease at the time of surgical treatment. Long-term survivals of 5.5 years and 5 years were achieved after local excision and pancreaticoduodenectomy, respectively.
Conclusions: Long-term survival is possible after local excision or pancreaticoduodenectomy.