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Molecular Profile SMARCB1 negative
Therapy Tazemetostat
Indication/Tumor Type epithelioid sarcoma
Response Type predicted - sensitive

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Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
SMARCB1 negative epithelioid sarcoma predicted - sensitive Tazemetostat Case Reports/Case Series Actionable In a Phase I trial, Tazemetostat (EPZ-6438) demonstrated safety and preliminary efficacy, resulted durable stable disease in 2 patients with SMARCB1-negative epithelioid sarcoma (PMID: 29650362; NCT01897571). 29650362
SMARCB1 negative epithelioid sarcoma predicted - sensitive Tazemetostat Phase II Actionable In a Phase II trial, Tazverik (tazemetostat) treatment resulted in an objective response rate of 15% (9/62) and a disease control rate of 26% (16/62) in patients with locally advanced or metastatic SMARCB1 (INI1)-negative epithelioid sarcoma, with a median duration of response not reached and a median overall survival of 82.4 weeks (J Clin Oncol 37, no. 15_suppl (May 20, 2019) 11003-11003, NCT02601950). detail...
SMARCB1 negative epithelioid sarcoma 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...
(29650362) Tazemetostat, an EZH2 inhibitor, in relapsed or refractory B-cell non-Hodgkin lymphoma and advanced solid tumours: a first-in-human, open-label, phase 1 study. Full reference...
Safety and efficacy of tazemetostat, a first-in-class EZH2 inhibitor, in patients (pts) with epithelioid sarcoma (ES) (NCT02601950). Full reference...