Autosomal dominantly inherited pathogenic germline variants have been implicated in the pathogenesis of ccRCC, such as in the VHL, c-Met, BaP1, and Pbrm1 genes [8,9,10], in which pathogenic variants in VHL have been identified as the leading cause of ccRCC pathogenesis [11]. This evidence concerns the gene BAP1 and nonpapillary renal cell carcinoma.