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An aggressive malignant neoplasm of uncertain differentiation, characterized by the presence of epithelioid cells forming nodular patterns. The nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern. It usually occurs in young adults. The most common sites of involvement are the extremities (distal-type epithelioid sarcoma), and less frequently the pelvis, perineum, and genital organs (proximal-type epithelioid sarcoma).
Biomarker and diagnostic research for epithelioid sarcoma has been reported in the published literature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
11 clinical trials registered, 5 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 5 PHASE2, 4 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT07089992](https://clinicaltrials.gov/study/NCT07089992) |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 6:53 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
A Study of Pembrolizumab in People With Ultra-Rare Sarcomas |
PHASE2 |
Memorial Sloan Kettering Cancer Center |
RECRUITING |
[NCT06625190](https://clinicaltrials.gov/study/NCT06625190) | Alpha/Beta T and B Cell Depletion With Zoledronic Acid for Solid Tumors | PHASE1 | University of Florida | RECRUITING |
[NCT06277154](https://clinicaltrials.gov/study/NCT06277154) | MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma | PHASE2 | HRYZ Biotech Co. | RECRUITING |
[NCT04390737](https://clinicaltrials.gov/study/NCT04390737) | Evaluate the Safety and Clinical Activity of HH2853 | PHASE1 | Haihe Biopharma Co., Ltd. | RECRUITING |
[NCT03967834](https://clinicaltrials.gov/study/NCT03967834) | Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group | NA | Institut Claudius Regaud | RECRUITING |
93 publications have been identified in PubMed for epithelioid sarcoma. Research spans Case Report / Case Series (54%), Basic Science / Preclinical (13%), and Epidemiology / Natural History (10%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 50 | 54% |
Laboratory research | 12 | 13% |
Disease patterns and progression | 9 | 10% |
Research summaries | 8 | 9% |
Clinical study results | 6 | 6% |
New treatment approaches | 5 | 5% |
Testing and diagnosis research | 3 | 3% |
Raeisi N (2026). [PMID: 40583176](https://pubmed.ncbi.nlm.nih.gov/40583176/). *Clinical nuclear medicine*. [Case Report / Case Series]
Oyama Y (2026). [PMID: 40781209](https://pubmed.ncbi.nlm.nih.gov/40781209/). *Medical molecular morphology*. [Case Report / Case Series]
Steubing Y (2026). [PMID: 41704273](https://pubmed.ncbi.nlm.nih.gov/41704273/). *Innovative surgical sciences*. [Review / Meta-Analysis]
Gambichler T (2026). [PMID: 41284360](https://pubmed.ncbi.nlm.nih.gov/41284360/). *Clinical and experimental dermatology*. [Review / Meta-Analysis]
Gorbunov S (2026). [PMID: 41898517](https://pubmed.ncbi.nlm.nih.gov/41898517/). *Int J Mol Sci*. [Basic Science / Preclinical]
Wang W (2026). [PMID: 42130619](https://pubmed.ncbi.nlm.nih.gov/42130619/). *Front Oncol*. [Case Report / Case Series]
Yeo B (2026). [PMID: 41623002](https://pubmed.ncbi.nlm.nih.gov/41623002/). *Current medical imaging*. [Case Report / Case Series]
Sobczuk P (2026). [PMID: 41874585](https://pubmed.ncbi.nlm.nih.gov/41874585/). *Curr Opin Oncol*. [Review / Meta-Analysis]
Obeidat A (2026). [PMID: 41731748](https://pubmed.ncbi.nlm.nih.gov/41731748/). *Medicine*. [Epidemiology / Natural History]
Kasyupa FE (2026). [PMID: 41797738](https://pubmed.ncbi.nlm.nih.gov/41797738/). *Clinical case reports*. [Case Report / Case Series]
AI-curated news mentioning epithelioid sarcoma
Updated Mar 24, 2026
New research highlights the therapeutic vulnerabilities associated with SMARCB1 loss in epithelioid sarcoma. This study provides insights that could inform future treatment strategies for this rare cancer.
Ipsen withdraws Tazverik from the market for follicular lymphoma and epithelioid sarcoma due to emerging data indicating an increased risk of secondary malignancies. This decision impacts ongoing treatment options for patients with these cancers.