title: Ampullary somatostatinoma in a patient with von Recklinghausen's disease.
abstract: We report a case of somatostatinoma of the ampulla of Vater associated with von Recklinghausen's disease in a 44-year-old woman. On admission the patient was jaundiced, and percutaneous Cholangio-drainage was performed. Cholangiography revealed stenosis of the common bile duct at the lower end Duodenoscopy showed a yellowish tumor of the ampulla of Vater, and the biopsy specimens showed no malignant cells. Pylorus-preserving pancreaticoduo-denectomy was performed. Histologically, the tumor was composed of small round cells with a solid or trabecular pattern and with multiple psammoma bodies. Immunohistochemical examination showed that the tumor cells stained for somatostatin. Genomic examination showed neither K-ras nor p53 gene mutations of the resected specimen.
authors.affiliation: Department of Surgery, Shimizu Kosei Hospital, Japan.
authors.forename: O
authors.initials: O
authors.lastname: Kainuma
authors.forename: Y
authors.initials: Y
authors.lastname: Ito
authors.forename: T
authors.initials: T
authors.lastname: Taniguchi
authors.forename: T
authors.initials: T
authors.lastname: Shimizu
authors.forename: H
authors.initials: H
authors.lastname: Nakada
authors.forename: Y
authors.initials: Y
authors.lastname: Date
authors.forename: T
authors.initials: T
authors.lastname: Hara
mesh_terms: D000328:Adult; D014670:Ampulla of Vater; D003138:Common Bile Duct Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005260:Female; D016158:Genes, p53 / Q000235:genetics; D011905:Genes, ras / Q000235:genetics; D006801:Humans; D009456:Neurofibromatosis 1 / Q000150:complications*; D016133:Polymerase Chain Reaction; D013005:Somatostatinoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery