title: Esophagogastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas in von Hippel-Lindau disease.
abstract: 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.
authors: Department of Gastroenterology, Osaka City University Medical School, Osaka, Japan.
authors: Masatsugu
authors: M
authors: Okuyama
authors: Yasuhiro
authors: Y
authors: Fujiwara
authors: Tsuyoshi
authors: T
authors: Hayakawa
authors: Masatsugu
authors: M
authors: Shiba
authors: Toshio
authors: T
authors: Watanabe
authors: Kazunari
authors: K
authors: Tominaga
authors: Akihiro
authors: A
authors: Tamori
authors: Nobuhide
authors: N
authors: Oshitani
authors: Kazuhide
authors: K
authors: Higuchi
authors: Takayuki
authors: T
authors: Matsumoto
authors: Kenji
authors: K
authors: Nakamura
authors: Kenichi
authors: K
authors: Wakasa
authors: Kazuhiro
authors: K
authors: Hirohashi
authors: Shingo
authors: S
authors: Ashida
authors: Taro
authors: T
authors: Shuin
authors: Tetsuo
authors: T
authors: Arakawa
mesh_terms: 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*
chemical_list: C497442:HIF1A protein, human; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D014157:Transcription Factors; D042461:Vascular Endothelial Growth Factor A