title: Periampullary neoplasms in von Recklinghausen's disease.
abstract: 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.
authors.affiliation: Department of Surgery, Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: A
authors.initials: A
authors.lastname: Klein
authors.forename: J
authors.initials: J
authors.lastname: Clemens
authors.forename: J
authors.initials: J
authors.lastname: Cameron
mesh_terms: D000328:Adult; D014670:Ampulla of Vater; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D003138:Common Bile Duct Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*