ORPHA:293202
Epithelioid sarcoma
Clinical definition (Orphanet)
A rare, soft tissue tumor characterized by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body.
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Is anyone studying this?
5,801
5,801 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=183) is 38.
5,801 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).
3,681 in the last 10 years · low confidence
Is a treatment being tested?
27
trials for this specific condition
27 interventional trials matched this specific condition name; 5 currently recruiting in our sample. 1,629 trials are registered for sarcoma, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 26 July 2026
1,629
trials for sarcoma, the broader category this belongs to
Trials registered for a broader category may or may not enrol people with this specific subtype — eligibility criteria vary, and the trial record often doesn't say. Worth raising with a clinician. How we count trials.
27 interventional trials — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 93.1th percentile).
low confidence · 93.1th percentile (trials denominator)
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Who's working on it?
1,170
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Wang J5 papers · 2026
Department of Bone and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.
Papers in Europe PMC - 02Kawai A4 papers · 2026
Department of Musculoskeletal Oncology and Rehabilitation, National Cancer Centre Hospital, Tokyo 104-0045, Japan.
Papers in Europe PMC - 03Liu Y4 papers · 2025
Department of Pathology, Sichuan Clinical Research Center for Cancer, Sichuan Cancer Hospital and Institute, Sichuan Cancer Center, Affiliated Cancer Hospital of University of Electronic Science and Technology of China, Chengdu, China. liuyang_sccancer@sina.com.
Papers in Europe PMC - 04Wang B4 papers · 2026
Department of Pathology, the People's Hospital of Bortala Mongolian Autonomous Prefecture, Bole, China.
Papers in Europe PMC - 05Wang X4 papers · 2026
Department of Bone and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.
Papers in Europe PMC - 06Yang J4 papers · 2026
Department of Cytopathology, Ningbo Clinical Pathology Diagnosis Center, Zhejiang, China.
Papers in Europe PMC - 07Yang Y4 papers · 2025
Department of Immunotherapy, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.
Papers in Europe PMC - 08Zhang P4 papers · 2025
Department of Bone and Soft Tissue, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.
Papers in Europe PMC - 09Chen Y3 papers · 2025
Department of Pathology, The Eighth Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Papers in Europe PMC - 10Li J3 papers · 2026
Department of Pathology, Sichuan Clinical Research Center for Cancer, Sichuan Cancer Hospital and Institute, Sichuan Cancer Center, Affiliated Cancer Hospital of University of Electronic Science and Technology of China, Chengdu, China.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
27 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT04390737·Evaluate the Safety and Clinical Activity of HH2853
- NCT06625190·Alpha/Beta T and B Cell Depletion With Zoledronic Acid for Solid Tumors
- NCT06444880·Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient Malignancies
- NCT03967834·Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group
- NCT06277154·MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma
Observational and natural-history studies
3 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored below but are not added to the query string.
"Epithelioid sarcoma" OR "epithelioid cell sarcoma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Epithelioid sarcoma" OR "epithelioid cell sarcoma"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 27 interventional · 3 observational · 3 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): UMLS:C0205944 NCIT:C3714
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: ES
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
- Publication count (5801) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity