The recognition of hereditary forms of renal cancer and the development of high-throughput genetic analysis have led to the identification of genes responsible for familial renal epithelial tumors of differing histologies and cytogenetic features. Some of these genes (VHL) are known to have an important role in sporadic renal neoplasia. This article describes the various epithelial renal tumors most commonly encountered by the urologist, the molecular and cytogenetic distinctions between them, and the hereditary syndromes that predispose to these tumors. Consideration of these syndromes is important for proper treatment when one encounters patients with multiple renal tumors, tumors at an early age of onset, or patients with a positive family history of renal cell carcinoma.
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