Epithelioid sarcoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-EPITHELIOID-SARCOMA |
|---|---|
| Type | Disease |
| Aliases | ESepithelioid sarcoma distal typeepithelioid sarcoma proximal typeЕпітеліоїдна саркома |
| Status | reviewed 2026-05-04 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-ESMO-SARCOMA-2024 SRC-EZH202-GOUNDER-2020 SRC-NCCN-SARCOMA |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8804/3 |
|---|---|
| icd_o_3_topography | ["C49"] |
| icd_10 | C49 |
| who_classification | WHO Soft Tissue Tumours 5th ed (2020): Epithelioid sarcoma |
Notes
Epithelioid sarcoma is defined by SMARCB1/INI1 loss and has historically poor prognosis in the proximal/aggressive variant. Tazemetostat (EZH2 inhibitor) is FDA-approved (2020) for metastatic or locally advanced epithelioid sarcoma not eligible for complete resection — the first and only FDA-approved targeted therapy. INI1 loss (detected by IHC) is the companion diagnostic. ORR ~15% in EZH-202 but durable responses seen. Note: tazemetostat is also approved for EZH2-mutant follicular lymphoma (different indication/biomarker). Distal-type has better prognosis than proximal-type; lymph node metastases are common (30%).
Used By
Actionability
BMA-SMARCB1-EPITHELIOID-SARCOMA- Tazemetostat (EPZ-6438), an EZH2 methyltransferase inhibitor, is FDA-approved (January 20...
Algorithms
ALGO-EPITHELIOID-SARCOMA-1L- ALGO-EPITHELIOID-SARCOMA-1L
Indication
IND-EPITHELIOID-SARCOMA-1L-TAZEMETOSTAT- IND-EPITHELIOID-SARCOMA-1L-TAZEMETOSTAT
Questionnaires
QUEST-EPITHELIOID-SARCOMA-1L-STUB- Epithelioid sarcoma — first line