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.
MeSH terms
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Angiography
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Arteries
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Arteriovenous Fistula / complications*
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Colon / blood supply*
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Cystadenoma, Serous / blood supply*
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Cystadenoma, Serous / diagnostic imaging
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Cystadenoma, Serous / etiology
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Cystadenoma, Serous / metabolism
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Endoscopy, Digestive System
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Esophageal and Gastric Varices / diagnosis
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Esophageal and Gastric Varices / etiology*
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Esophageal and Gastric Varices / pathology
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Esophageal and Gastric Varices / therapy
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Humans
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Hypoxia-Inducible Factor 1, alpha Subunit
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Immunohistochemistry
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Male
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Middle Aged
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Pancreatic Neoplasms / blood supply*
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Pancreatic Neoplasms / diagnostic imaging
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Pancreatic Neoplasms / etiology
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Pancreatic Neoplasms / metabolism
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Portal Vein*
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Rupture, Spontaneous / therapy
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Sclerotherapy
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Tomography, X-Ray Computed
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Transcription Factors / metabolism
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Vascular Endothelial Growth Factor A / metabolism
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von Hippel-Lindau Disease / complications*
Substances
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HIF1A protein, human
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Hypoxia-Inducible Factor 1, alpha Subunit
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Transcription Factors
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Vascular Endothelial Growth Factor A