Since the finding that germline-inactivating BAP1 mutations predispose MPM [135], increasing evidence has confirmed this discovery, showing autosomal dominant germline mutations in the BAP1 gene in almost all MPM patients 50 years old or younger with a family history of mesothelioma and/or uveal melanoma (UVM) or clear cell renal cell carcinoma (ccRCC) [136]. This evidence concerns the gene BAP1 and nonpapillary renal cell carcinoma.