SMARCB1 and telomere syndrome: Medications currently under investigation for treatment of STS with novel mechanisms of action include γ-secretase inhibitors (Notch and WNT/β-catenin pathway) for treatment of DT, MDM2 inhibitors targeting P53 for treatment of LPS given high expression of MDM2 in this STS subtype, and BRD9 inhibitors targeting ncBAF complex for treatment of synovial sarcoma and other SMARCB1-loss tumors.