Direct evidence of the inherited predisposition to RCC is provided by a number of rare cancer syndromes with defined germline mutations in 11 genes (BAP1, FLCN, FH, MET, PTEN, SDHB, SDHC, SDHD, TSC1, TSC2, and VHL), associated with the development of different RCC subtypes [28]. Here, TSC2 is linked to renal cell adenocarcinoma.