RCC patients with a germline FH or SDH mutation are predisposed to developing additional syndromic features; FHRCC patients can develop cutaneous and uterine leiomyomas [9] comprising a genetic syndrome called hereditary leiomyomatosis and RCC (HLRCC); SDHRCC patients have a higher risk of developing pheochromocytomas/paragangliomas [10] and gastrointestinal stromal tumors [11]. This evidence concerns the gene SDHB and renal cell carcinoma.