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Okuyama, M. et al.

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.[1]

References

Esophagogastric varices due to arterioportal shunt in a serous cystadenoma of the pancreas in von Hippel-Lindau disease. Okuyama, M., Fujiwara, Y., Hayakawa, T., Shiba, M., Watanabe, T., Tominaga, K., Tamori, A., Oshitani, N., Higuchi, K., Matsumoto, T., Nakamura, K., Wakasa, K., Hirohashi, K., Ashida, S., Shuin, T., Arakawa, T. Dig. Dis. Sci. (2003) [Pubmed]

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