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Molecular Profile | SMARCB1 negative |
Therapy | Tazemetostat |
Indication/Tumor Type | chordoma |
Response Type | predicted - sensitive |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
SMARCB1 negative | chordoma | predicted - sensitive | Tazemetostat | Phase I | Actionable | In a Phase I trial, Tazemetostat (EPZ-6438) was well-tolerated and demonstrated anti-tumor activity in pediatric patients with SMARCB1 (INI)-deficient tumors, including complete or partial responses in patients with epithelioid sarcoma (n=1), chordoma (n=2), and atypical teratoid rhaboid tumor (n=1) (AACR-NCI-EORTC Int Conference on Molecular Targets and Cancer Therapeutics 2017, A175; NCT02601937). | detail... |
PubMed Id | Reference Title | Details |
---|---|---|
A175 / 175 - Phase 1 study of the EZH2 inhibitor, tazemetostat, in children with relapsed or refractory INI1-negative tumors including rhabdoid tumors, epithelioid sarcoma, chordoma, and synovial sarcoma | Full reference... |