Islet cell tumor in von Hippel-Lindau disease.
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.
Mizuno
N
N
Department of Internal Medicine II, Nagoya University School of Medicine.
Naruse
S
S
Kitagawa
M
M
Ishiguro
H
H
Nakae
Y
Y
Ieda
H
H
Iizuka
N
N
Ito
O
O
Seki
Y
Y
Ko
S B
SB
Yoshikawa
T
T
Hayakawa
T
T
Sano
T
T
Kamiya
J
J
Nimura
Y
Y
Nagasaka
T
T
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
D014408:Biomarkers, Tumor; D053379:Chromogranin A; D002864:Chromogranins; D009418:S100 Proteins; D010191:Pancreatic Polypeptide