Tazemetostat monotherapy (advanced epithelioid sarcoma, SMARCB1/INI1-deficient)
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| Components | DRUG-TAZEMETOSTAT |
|---|
| ID | REG-TAZEMETOSTAT-EPITHELIOID-SARCOMA |
|---|---|
| Type | Regimen |
| Status | reviewed 2026-05-04 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-ESMO-SARCOMA-2024 SRC-EZH202-GOUNDER-2020 |
Notes
EZH-202 trial (Gounder 2020 NEJM): tazemetostat in advanced epithelioid sarcoma with SMARCB1/INI1 deficiency — ORR ~15%, but responses were durable (DoR not reached in responders). Given the lack of alternatives in this ultra-rare sarcoma, even modest ORR with durable response is clinically meaningful. FDA approved January 2020 — first and only FDA-approved targeted therapy for epithelioid sarcoma. Secondary malignancy risk (T-LBL/AML) was observed in pediatric sarcoma trials; ongoing surveillance required.
Used By
Indication
IND-EPITHELIOID-SARCOMA-1L-TAZEMETOSTAT- IND-EPITHELIOID-SARCOMA-1L-TAZEMETOSTAT