Although an efficient antitumoral activity of the anti-EZH2 tazemetostat has already been reported in one SMARCB1/INI1 negative chordoma [28], there are no data concerning anti-EZH2 drugs on PBRM1-mutated chordoma, which is one of the most common genetic type [7]. This evidence concerns the gene PBRM1 and chordoma.