Although BHD gene mutations, including folliculin, have been found in 80% of BHD strains, chromophobe RCC mutations are much rarer [68]. TSC has been linked to germline TSC1 (9q34) hamartin encoding or TSC2 (16p13.3) encoding tuberin mutations and affected patients have an increased risk of developing kidney tumors such as ccRCC, papillary RCC, and chromophobe RCC [69]. Here, TSC1 is linked to nonpapillary renal cell carcinoma.