Epithelioid sarcoma

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Section editor
Elizabeth J. Davis, MD
Vanderbilt University
Nashville, TN, USA

Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens.

1 regimens on this page
1 variants on this page


Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.


All lines of therapy

Tazemetostat monotherapy


FDA-recommended dose
Study Dates of enrollment Evidence
Gounder et al. 2020 (EZH-202) 2015-2017 Phase 2 (RT)

Biomarker eligibility criteria

One or both of the following:

  • Loss of INI1 expression by IHC
  • Biallelic SMARCB1 alterations

Targeted therapy

28-day cycles


  1. EZH-202: Gounder M, Schöffski P, Jones RL, Agulnik M, Cote GM, Villalobos VM, Attia S, Chugh R, Chen TW, Jahan T, Loggers ET, Gupta A, Italiano A, Demetri GD, Ratan R, Davis LE, Mir O, Dileo P, Van Tine BA, Pressey JG, Lingaraj T, Rajarethinam A, Sierra L, Agarwal S, Stacchiotti S. Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study. Lancet Oncol. 2020 Nov;21(11):1423-1432. Epub 2020 Oct 6. link to original article contains dosing details in abstract PubMed NCT02601950