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: 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
• Journal: Digestive diseases and sciences (Dig Dis Sci) Vol. 48 Issue 10 Pg. 1948-54 (Oct 2003) ISSN: 0163-2116 [Print] United States
• PMID: 14627339 (Publication Type: Case Reports, Journal Article)

Chemical References

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

Topics

• Angiography
• Arteries
• Arteriovenous Fistula (complications)
• Colon (blood supply)
• Cystadenoma, Serous (blood supply, diagnostic imaging, etiology, metabolism)
• Endoscopy, Digestive System
• Esophageal and Gastric Varices (diagnosis, etiology, pathology, therapy)
• Humans
• Hypoxia-Inducible Factor 1, alpha Subunit
• Immunohistochemistry
• Male
• Middle Aged
• Pancreatic Neoplasms (blood supply, diagnostic imaging, etiology, 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)