Genetic disorders and renal cell carcinoma.

The study of hereditary RCC syndromes continues to provide significant insight into the pathways that are involved in renal cell tumorigenesis. The clinician should maintain a high level of suspicion for genetic disorders when patients present with early-onset or mult-focal RCC. Recognition of familial syndromes will facilitate the institution of parenchymal sparing measures, as well as appropriate screening and intervention for associated nonrenal manifestations.