Esophagogastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas in von Hippel-Lindau disease

Masatsugu Okuyama; Yasuhiro Fujiwara; Tsuyoshi Hayakawa; Masatsugu Shiba; Toshio Watanabe; Kazunari Tominaga; Akihiro Tamori; Nobuhide Oshitani; Kazuhide Higuchi; Takayuki Matsumoto; Kenji Nakamura; Kenichi Wakasa; Kazuhiro Hirohashi; Shingo Ashida; Taro Shuin; Tetsuo Arakawa

doi:10.1023/a:1026262019552

Esophagogastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas in von Hippel-Lindau disease

Dig Dis Sci. 2003 Oct;48(10):1948-54. doi: 10.1023/a:1026262019552.

Authors

Masatsugu Okuyama¹, Yasuhiro Fujiwara, Tsuyoshi Hayakawa, Masatsugu Shiba, Toshio Watanabe, Kazunari Tominaga, Akihiro Tamori, Nobuhide Oshitani, Kazuhide Higuchi, Takayuki Matsumoto, Kenji Nakamura, Kenichi Wakasa, Kazuhiro Hirohashi, Shingo Ashida, Taro Shuin, Tetsuo Arakawa

Affiliation

¹ Department of Gastroenterology, Osaka City University Medical School, Osaka, Japan.

PMID: 14627339
DOI: 10.1023/a:1026262019552

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.

Publication types

Case Reports

MeSH terms

Angiography
Arteries
Arteriovenous Fistula / complications*
Colon / blood supply*
Cystadenoma, Serous / blood supply*
Cystadenoma, Serous / diagnostic imaging
Cystadenoma, Serous / etiology
Cystadenoma, Serous / metabolism
Endoscopy, Digestive System
Esophageal and Gastric Varices / diagnosis
Esophageal and Gastric Varices / etiology*
Esophageal and Gastric Varices / pathology
Esophageal and Gastric Varices / therapy
Humans
Hypoxia-Inducible Factor 1, alpha Subunit
Immunohistochemistry
Male
Middle Aged
Pancreatic Neoplasms / blood supply*
Pancreatic Neoplasms / diagnostic imaging
Pancreatic Neoplasms / etiology
Pancreatic Neoplasms / metabolism
Portal Vein*
Rupture, Spontaneous / therapy
Sclerotherapy
Tomography, X-Ray Computed
Transcription Factors / metabolism
Vascular Endothelial Growth Factor A / metabolism
von Hippel-Lindau Disease / complications*

Substances

HIF1A protein, human
Hypoxia-Inducible Factor 1, alpha Subunit
Transcription Factors
Vascular Endothelial Growth Factor A