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ORPHA:370334

Extraskeletal Ewing sarcoma

low confidenceDisorder

Also known as: EOE · Extraosseous Ewing sarcoma · Extraosseous Ewing tumor · Extraskeletal Ewing tumor

Publications

1,160

Trials

6

Interventional, condition-specific

Researchers

1,088

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Extraskeletal Ewing sarcoma is a rare, poorly differentiated, highly malignant, soft tissue tumor, derived from neuroectoderm, that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations, with the most common being: chest wall, paravertebral region, abdominopelvic area (with predilection for the retroperitoneal space), gluteal region and lower extremities. Clinical presentation is highly variable and depends on tumor localization. Local recurrence is common and metastatic disease most frequently involves the bones and lungs.

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.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (11)

Extra-osseous Ewing's sarcoma · extraosseous Ewing sarcoma · extraosseous Ewing tumor · extraosseous Ewing tumour · extraosseous Ewing's sarcoma · extraosseous Ewing's tumor · extraosseous Ewing's tumour · extraskeletal Ewing sarcoma · extraskeletal Ewing tumor · extraskeletal Ewing tumour · extraskeletal Ewing's sarcoma

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    1,160 matched papers (600 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    6 matched on ClinicalTrials.gov (1 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

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.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

1,160

1,160 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

1,160 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

600 in the last 10 years · low confidence

Phrase hits: 1,160 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,088

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.

  1. 01
    Zhang S4 papers · 2026

    The First Clinical Medical College of Guangdong Medical University, Zhanjiang.

    Papers in Europe PMC
  2. 02
    Li W3 papers · 2025

    Department of Medical Oncology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, China.

    Papers in Europe PMC
  3. 03
    Li Y3 papers · 2025

    Ultrasound Medicine Center, Lanzhou University Second Hospital, Chengguan District, Lanzhou, 730030, Gansu, China.

    Papers in Europe PMC
  4. 04
    Liu X3 papers · 2025

    Department of Pathology, the Second People's Hospital of Shenzhen, Guangdong Province (the First Affiliated Hospital of Shenzhen University), Shenzhen 518000, China.

    Papers in Europe PMC
  5. 05
    Singh A3 papers · 2025

    Department of Radiation Oncology, RCC JIPMER, Puducherry, India.

    Papers in Europe PMC
  6. 06
    Wu Y3 papers · 2025

    Department of Radiology, The Affiliated Hospital of Zunyi Medical University, Zunyi, China.

    Papers in Europe PMC
  7. 07
    Zhang Y3 papers · 2025

    Department of Radiology, The Second Affiliated Hospital of Dalian Medical University, 467 Zhong Shan Road, Dalian, 116023, China.

    Papers in Europe PMC
  8. 08
    Abulihya M2 papers · 2024

    Department of Pathology, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine.

    Papers in Europe PMC
  9. 09
    Alizadehasl A2 papers · 2024

    Cardio-Oncology Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.

    Papers in Europe PMC
  10. 10
    Allali N2 papers · 2026

    Radiology Department Mother and Child Hospital Ibn Sina, Rabat, Morocco.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

6

interventional trials for this specific condition

6 interventional trials matched this specific condition name; 1 currently recruiting in our sample. 161 trials are registered for Ewing sarcoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

6 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 90.1th percentile).

low confidence · 90.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

6 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: Ewing sarcoma

161

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (1)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Extraskeletal Ewing sarcoma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This 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 on the overview but are not added to the query string.

"Extraskeletal Ewing sarcoma" OR "Extraosseous Ewing sarcoma" OR "Extraosseous Ewing tumor" OR "Extraskeletal Ewing tumor" OR "Extra-osseous Ewing's sarcoma" OR "extraosseous Ewing tumour" OR "extraosseous Ewing's sarcoma" OR "extraosseous Ewing's tumor" OR "extraosseous Ewing's tumour" OR "extraskeletal Ewing tumour" OR "extraskeletal Ewing's sarcoma"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Extraskeletal Ewing sarcoma" OR "Extraosseous Ewing sarcoma" OR "Extraosseous Ewing tumor" OR "Extraskeletal Ewing tumor" OR "Extra-osseous Ewing's sarcoma" OR "extraosseous Ewing tumour" OR "extraosseous Ewing's sarcoma" OR "extraosseous Ewing's tumor" OR "extraosseous Ewing's tumour" OR "extraskeletal Ewing tumour" OR "extraskeletal Ewing's 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: 6 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"Ewing sarcoma"

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: EOE

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 (1160) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-27T14:56:06.641Z