von Hippel-Lindau Tumor Suppressor, Hypoxia-Inducible Factor-1, and Tumor Vascularization

Part of the Cancer Genetics book series (CANGENETICS)


von Hippel–Lindau (VHL) disease is an autosomal dominant, familial cancer syndrome that is characterized by the development of various benign and malignant tumors. The most frequent tumors are hemangioblastoma (HB) in the central nervous system (CNS), pheochromocytoma (Pheo), and renal-cell carcinoma of the clear-cell type (RCC). VHL families have been subdivided into those with a low risk of pheochromocytoma (type 1 VHL disease) and those with a high risk of pheochromocytoma (type 2 VHL disease). VHL type 2 disease is further classified into three categories: type 2A, type 2B, and type 2C. Type 2A VHL disease has pheochromocytoma and hemangioblastoma in the CNS, but not RCC. Type 2B exhibits pheochromocytoma, RCC, and hemangioblastoma. Type 2C disease has only pheochromocytoma, without hemangioblastoma or RCC.


Vascular Endothelial Growth Factor Atypical Protein Kinase 


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© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Departments of Pediatrics, Medicine, Oncology, and Radiation OncologyThe Johns Hopkins University School of MedicineBaltimoreUSA

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