Currently, some agents include: (1) EZH2 inhibitors, which prevent SMARCB1/INI1-deficient neoplasms progression and have been proved to sensitize neoplastic cells to the effects of radiation therapy; (2) CDK4 inhibitors, which inhibit tumor cell growth by G1 arrest; (3) aurora-A-kinase inhibitors, inhibits aurora A; (4) histone deacetylase inhibitors (HDACi), with a similar mechanism of action to the EZH2 inhibitors; and (5) DNA methyltransferase inhibitors [33,34]. Here, SMARCB1 is linked to neoplasm.