Established increased risk of renal cancer and/or melanoma is observed with BAP1 mutations responsible for BAP1-TPDS, PTEN-AKT1/2-PIK3CA induced PTEN-opathies [90], as well as mTOR signaling syndromes such as TSC1/2 tuberous sclerosis complex [91]. Here, MTOR is linked to renal carcinoma.