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    Association details:
    Biomarker:SMARCB1 negative
    Cancer:Chordoma
    Drug:Tazverik (tazemetostat) (EZH2 inhibitor)
    Direction:Sensitive
    Evidence:
    Evidence Level:
    Sensitive: C2 – Inclusion Criteria
    Go to data
    Source:
    clinicaltrials.gov
    Title:

    A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma

    Excerpt:
    ...Other INI1-negative malignant tumors (e.g., dedifferentiated chordoma) with Sponsor approval...
    Trial ID:
    NCT02601937
    Evidence Level:
    Sensitive: C3 – Early Trials
    Source:
    AACR 2018
    Title:

    Abstract A175: 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

    Excerpt:
    Tazemetostat was generally well tolerated in children and showed promising antitumor activity including CRs in pts with INI1-negative tumors.
    DOI:
    10.1158/1535-7163.TARG-17-A175
    Trial ID:
    NCT02601937