The newly identified genes are polybromo-1 (PBRM1), AT-rich interactive domain-containing protein 1A (ARID1A), BRCA1 associated protein-1 (BAP1), SET domain-containing 2 (SETD2), and lysine- (K-) specific demethylase 5C (KDM5C) [89–93]. PBRM1 mutations are found in up to 41% of ccRCC, making it the second most mutated gene after VHL [90]. PBRM1, BAP1, and SETD2 are all located near the 3p21 region and, similar to VHL, are proposed to be inactivated through the Knudson two-hit model [94]. The gene discussed is ARID1A; the disease is nonpapillary renal cell carcinoma.