This hypothesis has recently been supported by Haugh et al who by review of the literature reported twenty cases of RCC in 215 patients with pathogenic BAP1-variants corresponding to an estimated penetrance for RCC of 9% [19].Microphthalmia-associated transcription factor (MITF) regulates the expression of genes involved in the cell cycle, cellular proliferation and differentiation [20]. The gene discussed is MITF; the disease is renal cell carcinoma.