RecruitingPhase 1 / Phase 2NCT06239272
Oncology
Epithelioid Sarcoma
Also known as ES, SMARCB1/INI1 loss sarcoma, proximal epithelioid sarcoma
Epithelioid sarcoma is an ultrarare malignant soft tissue tumour defined by loss of SMARCB1 (INI1) expression due to biallelic inactivation of the SMARCB1 tumour suppressor gene, which encodes a core subunit of the SWI/SNF chromatin remodel
ORPHA:31167 ↗Gene SMARCB1Prevalence Less than 1 in 1,000,000Onset Young adult (2nd–4th decade for classic type; older for proximal type)Sporadic somatic
13
studies recruiting now
as of 7 Sept 2026
72
studies registered in total
as of 7 Sept 2026
1
countries with a recruiting site
as of 7 Sept 2026
30 Jun 2026
most recent study posted
among recruiting studies
Recruiting trials
RecruitingPhase 2NCT07089992
A Study of Pembrolizumab in People With Ultra-Rare Sarcomas
RecruitingNCT07675967
Clonal Hematopoiesis Chemotherapy and Radiation Effects Study
RecruitingPhase 1 / Phase 2NCT06625190
Alpha/Beta T and B Cell Depletion With Zoledronic Acid for Solid Tumors
RecruitingPhase 2NCT07467772
Ph 2 Elacestrant in ER Positive Uterine Sarcomas
Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.
See all 13 recruiting studiesWhere recruiting studies are running
Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.
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About Epithelioid Sarcoma
Epithelioid sarcoma is an ultrarare malignant soft tissue tumour defined by loss of SMARCB1 (INI1) expression due to biallelic inactivation of the SMARCB1 tumour suppressor gene, which encodes a core subunit of the SWI/SNF chromatin remodelling complex. Two subtypes are recognised: the classic (distal) type presenting as a slow-growing nodule in the distal extremities of young adults, and the proximal type arising in axial locations with more aggressive behaviour. Loss of EZH2 antagonism resulting from SMARCB1 deficiency renders epithelioid sarcoma uniquely sensitive to EZH2 inhibition — a mechanism exploited by tazemetostat, the first approved therapy for this tumour.
Common clinical features
Slow-growing, firm nodule or plaque on the hand, wrist, or forearm (classic type) often initially mistaken for a benign lesionUlceration of overlying skin, particularly in distal extremity tumours, mimicking a chronic woundProximal type: deep-seated pelvic, perineal, or axial mass with more rapid growthRegional lymph node metastases, with lymphatic spread rates higher than most sarcomasBone invasion and periosteal involvement in advanced distal extremity casesPulmonary metastases as the most common site of distant diseaseMultifocal local recurrence along fascial planes after resection
From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.
Before you apply
Things trial teams commonly ask about for Epithelioid Sarcoma. Not eligibility rules; those are set by each study.
- Loss of INI1 (SMARCB1) expression by immunohistochemistry is the diagnostic and eligibility criterion for EZH2 inhibitor trials — pathology report must specifically document INI1-negative staining.
- Tazemetostat (FDA-approved for this indication) may affect eligibility for combinatorial EZH2 inhibitor trials; document prior exposure to EZH2 inhibitors including dates and response.
- Archival tumour tissue for central pathology review is typically required; ensure sufficient FFPE block material is available and arrange transfer of blocks or unstained sections early in the screening process.
Related conditions
Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).