Esophagogastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas in von Hippel-Lindau disease.
Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited disorder characterized by extensively vascularized tumors and cysts in specific organs. Angiogenesis is a striking future of VHL disease with its characteristic cysts and well-vascularized tumors. The hypervascular nature of VHL lesions has been linked to the overproduciton of vascular endothelial growth factor (VEGF) through increased expression of hypoxia inducible factor-1alpha (HIF-1alpha). Here we describe a rare case of VHL disease with esophageal and gastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas, which, upon immunohistochemical examination, exhibited HIF-1alpha and VEGF expression. Rupture of esophageal varices was successfully treated with endoscopic injection sclerotherapy.
Okuyama
Masatsugu
M
Department of Gastroenterology, Osaka City University Medical School, Osaka, Japan.
Fujiwara
Yasuhiro
Y
Hayakawa
Tsuyoshi
T
Shiba
Masatsugu
M
Watanabe
Toshio
T
Tominaga
Kazunari
K
Tamori
Akihiro
A
Oshitani
Nobuhide
N
Higuchi
Kazuhide
K
Matsumoto
Takayuki
T
Nakamura
Kenji
K
Wakasa
Kenichi
K
Hirohashi
Kazuhiro
K
Ashida
Shingo
S
Shuin
Taro
T
Arakawa
Tetsuo
T
D000792:Angiography; D001158:Arteries; D001164:Arteriovenous Fistula / Q000150:complications*; D003106:Colon / Q000098:blood supply*; D018293:Cystadenoma, Serous / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000209:etiology / Q000378:metabolism; D016145:Endoscopy, Digestive System; D004932:Esophageal and Gastric Varices / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000209:etiology / Q000378:metabolism; D011169:Portal Vein; D012422:Rupture, Spontaneous / Q000628:therapy; D015911:Sclerotherapy; D014057:Tomography, X-Ray Computed; D014157:Transcription Factors / Q000378:metabolism; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism; D006623:von Hippel-Lindau Disease / Q000150:complications*
C497442:HIF1A protein, human; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D014157:Transcription Factors; D042461:Vascular Endothelial Growth Factor A