Tazemetostat (Food and Drug Administration, FDA approved in Jan 2020), a specific histone-lysine N-methyltransferase enzyme inhibitor (enhancer of zeste homolog 2, EZH2) is the first targeted treatment for epithelioid sarcoma with a median progression-free survival of ~6 months and a median overall survival of ~1.5 years [6]. This evidence concerns the gene EZH2 and epithelioid sarcoma.