Given preclinical data demonstrating the antitumor activity of EZH2 inhibition in BAP1 mutant mesothelioma, a phase 2 clinical trial evaluated the safety and efficacy of tazemetostat, a selective oral EZH2 inhibitor, in patients with relapsed or refractory MPM (NCT02860286) [31,32]. This evidence concerns the gene BAP1 and mesothelioma.