Ampullary somatostatinoma in a patient with von Recklinghausen's disease.
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.
Kainuma
O
O
Department of Surgery, Shimizu Kosei Hospital, Japan.
Ito
Y
Y
Taniguchi
T
T
Shimizu
T
T
Nakada
H
H
Date
Y
Y
Hara
T
T
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