Hereditary renal cell carcinoma (RCC) has been estimated to account for 5% to 8% of all RCC cases, and extrarenal manifestations may present as early as 3 years of age (Table 1).1,2 RCC is a diverse set of cancers that originate from the renal parenchyma. Histologic classifications include clear cell, papillary, chromophobe, and translocation; rare subtypes include renal medullary and collecting duct. Hereditary familial RCC syndromes have yielded clues regarding the molecular pathogenesis of sporadic RCC and have served as a framework for the development of targeted therapies. With diverse presentations and incomplete penetrance of RCC, establishing screening guidelines for detecting early-onset RCC may help identify persons with germline mutations who have an increased risk of developing RCC.
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