title: A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1.
abstract: Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunction and was admitted to our hospital for further examination. An abdominal contrast-enhanced computed tomography scan demonstrated a 30-mm tumor in the head of the pancreas. The scan showed an invasion of the tumor into the duodenum, and biopsy under an endoscopic ultrasonography indicated that the tumor was a NET. A subtotal stomach-preserving pancreaticoduodenectomy was performed. Macroscopically, the pancreatic tumor was white and elastic hard. Microscopically, tumor cells were composed of ribbons, cords, and solid nests with an acinus-like structure. The tumor was diagnosed as NET G2 according to the WHO classification (2010). The product of the NF-1 gene, i.e., neurofibromin, was weakly positive in the tumor cells, suggesting that the tumor was induced by a mutation in the NF-1 gene. This is the seventh case of PNET arising in NF-1 patients worldwide.
authors: Department of Digestive and General Surgery, Shimane University Faculty of Medicine, Izumo, Shimane, Japan. nishiken@med.shimane-u.ac.jp
authors: Takeshi
authors: T
authors: Nishi
authors: Yasunari
authors: Y
authors: Kawabata
authors: Youko
authors: Y
authors: Hari
authors: Hiroshi
authors: H
authors: Imaoka
authors: Noriyoshi
authors: N
authors: Ishikawa
authors: Seiji
authors: S
authors: Yano
authors: Riruke
authors: R
authors: Maruyama
authors: Yoshitsugu
authors: Y
authors: Tajima
mesh_terms: D019160:Endosonography; D016514:Genes, Neurofibromatosis 1; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D016577:Pancreaticoduodenectomy