In addition to predisposing to RCC, germline BAP1 mutations predispose to mesothelioma (pleural and peritoneal), uveal melanoma and cutaneous melanoma, so the personal or family history of these features should lead to the suspicion of BAP1 tumour predisposition syndrome (BPTS) [31]. The gene discussed is BAP1; the disease is BAP1-related tumor predisposition syndrome.