Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Renal Cancer Pathogenesis

  • Harry A. Drabkin
  • Jeffrey Turner
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_6324-2

Characteristics

Conventional or Clear-Cell Renal Cell Carcinoma (ccRCC). Clear-cell renal cell carcinoma (ccRCC) accounts for approximately 75 % of all renal epithelial cancers, is the most frequent malignant neoplasm arising from the kidney, and is the most common cause of death in patients with von Hippel-Lindau disease (VHL; von Hippel-Lindau tumor suppressor gene), an autosomal dominant familial cancer syndrome. This syndrome is comprised of retinal angiomas, hemangioblastomas of the central nervous system, pheochromocytoma, endolymphatic sac tumors of the middle ear, serous cystadenomas and neuroendocrine tumors of the pancreas, papillary cystadenomas of the epididymis or broad ligament, and ccRCC. In von Hippel-Lindau disease, one VHL mutant allele is inherited. In a majority of cases, ccRCC arises from acquired mutation or silencing of the remaining wild-type allele. The use of tobacco (tobacco carcinogenesis) appears to be a major risk factor in patients with sporadic disease.

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Keywords

Renal Cell Carcinoma Thyroid Medullary Carcinoma Tuberous Sclerosis Complex Fumarate Hydratase Renal Oncocytomas 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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See Also

  1. (2012) Autosomal Dominant. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 323. doi:10.1007/978-3-642-16483-5_489Google Scholar
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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Division of Hematology-OncologyMedical University of South Carolina and the Hollings Cancer CenterCharlestonUSA
  2. 2.Compassionate Oncology Medical GroupLos AngelesUSA