The mechanism may involve the inhibition of neural differentiation genes, cell cycle inhibitors, and tumor suppressor factors by EZH2, while also reducing the expression of GLI1, Patch1, MYC.Tazemetostat is the first-in-class oral EZH2 inhibitor approved by the U.S. Food and Drug Administration (FDA) for the treatment of SMARCB1-deficient epithelioid sarcoma (23). This evidence concerns the gene SMARCB1 and epithelioid sarcoma.