The spectrum of tumor associations in BAP1 tumor predisposition syndrome has gradually expanded from uveal melanoma and mesothelioma to include early-onset clear cell RCC.3,4 For instance, in 1 study,5 13 RCC-affected individuals from 7 families were found to have pathogenic germline alterations involving BAP1. This evidence concerns the gene BAP1 and BAP1-related tumor predisposition syndrome.