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Renal Cancer Pathogenesis

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Encyclopedia of Cancer

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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Correspondence to Harry A. Drabkin .

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Drabkin, H.A., Turner, J. (2014). Renal Cancer Pathogenesis. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_6324-2

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