title: Islet cell tumor in von Hippel-Lindau disease.
abstract: We describe a 42-year-old man with von Hippel-Lindau disease and islet cell tumor of the pancreas. He had retinal and cerebellar hemangioblastomas. His sister had pheochromocytoma. A pancreatic tumor was detected by ultrasonography at his periodical medical checkup. Contrast enhanced computed tomography and abdominal angiography revealed a hypervascular tumor in the pancreatic head. Histological examination of the resected tumor revealed characteristics of islet cell tumor of the pancreas, which was positive for chromogranin-A, S-100 protein, and pancreatic polypeptide, but was negative for insulin, gastrin, glucagon, somatostatin, vasoactive intestinal peptide, serotonin, and adrenocorticotropic hormone.
authors.affiliation: Department of Internal Medicine II, Nagoya University School of Medicine.
authors.forename: N
authors.initials: N
authors.lastname: Mizuno
authors.forename: S
authors.initials: S
authors.lastname: Naruse
authors.forename: M
authors.initials: M
authors.lastname: Kitagawa
authors.forename: H
authors.initials: H
authors.lastname: Ishiguro
authors.forename: Y
authors.initials: Y
authors.lastname: Nakae
authors.forename: H
authors.initials: H
authors.lastname: Ieda
authors.forename: N
authors.initials: N
authors.lastname: Iizuka
authors.forename: O
authors.initials: O
authors.lastname: Ito
authors.forename: Y
authors.initials: Y
authors.lastname: Seki
authors.forename: S B
authors.initials: SB
authors.lastname: Ko
authors.forename: T
authors.initials: T
authors.lastname: Yoshikawa
authors.forename: T
authors.initials: T
authors.lastname: Hayakawa
authors.forename: T
authors.initials: T
authors.lastname: Sano
authors.forename: J
authors.initials: J
authors.lastname: Kamiya
authors.forename: Y
authors.initials: Y
authors.lastname: Nimura
authors.forename: T
authors.initials: T
authors.lastname: Nagasaka
mesh_terms: D007516:Adenoma, Islet Cell / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D000328:Adult; D014408:Biomarkers, Tumor / Q000097:blood; D002760:Cholangiopancreatography, Endoscopic Retrograde; D053379:Chromogranin A; D002864:Chromogranins / Q000097:blood; D019160:Endosonography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D010191:Pancreatic Polypeptide / Q000097:blood; D016577:Pancreaticoduodenectomy; D009418:S100 Proteins / Q000097:blood; D014057:Tomography, X-Ray Computed; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis
chemical_list: D014408:Biomarkers, Tumor; D053379:Chromogranin A; D002864:Chromogranins; D009418:S100 Proteins; D010191:Pancreatic Polypeptide