Periampullary neoplasms in von Recklinghausen's disease

A case of neurofibromatosis is reported in a patient who was initially seen with obstructive jaundice caused by a carcinoid tumor originating from the ampulla of Vater. An extensive review of the literature suggests that patients with von Recklinghausen's disease are at significant risk for periampullary neoplasms of neural-crest and non-neural-crest origin. The tendency of those tumors to arise from the ampulla of Vater has diagnostic and therapeutic implications.