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

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Cancer Genome and Tumor Microenvironment

Part of the book series: Cancer Genetics ((CANGENETICS))

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Abstract

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.

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Authors and Affiliations

  1. Departments of Pediatrics, Medicine, Oncology, and Radiation Oncology, The Johns Hopkins University School of Medicine, Broadway Research Building, Suite 671, Baltimore, MD, USA

    Huafeng Zhang & Gregg L. Semenza

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  1. Huafeng Zhang
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  2. Gregg L. Semenza
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  1. Children's Hospital of Philadelphia, Civic Center Blvd. 3615, Philadelphia, 19104, U.S.A.

    Andrei Thomas-Tikhonenko

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Zhang, H., Semenza, G.L. (2010). von Hippel-Lindau Tumor Suppressor, Hypoxia-Inducible Factor-1, and Tumor Vascularization. In: Thomas-Tikhonenko, A. (eds) Cancer Genome and Tumor Microenvironment. Cancer Genetics. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-0711-0_6

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