Recent advances in next-generation sequencing (NGS) and their implementation in large cohort of cancer patients (e.g., The Cancer Genome Atlas (TCGA) projects), have led to the identification of additional genes frequently mutated in ccRCC, such as PBRM1 (≈40–50%), SETD2 (12%), BAP1 (10%), and KDM5C (5%) [9,10,11]. This evidence concerns the gene PBRM1 and nonpapillary renal cell carcinoma.