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ORPHA:163699

Alveolar soft tissue sarcoma

low confidenceDisorder

Also known as: ASPS · Alveolar soft part sarcoma

Publications

2,885

Trials

35

Interventional, condition-specific

Researchers

1,372

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare soft tissue sarcoma characterized by a slowly growing, painless space-occupying lesion, composed of large, uniform, epithelioid cells arranged in solid nests and/or alveolar structures, separated by thin, sinusoidal vessels. The tumor mostly affects adolescents and young adults. Early metastasis, most commonly to the lung, bones, and brain, is a characteristic feature and relevant prognostic factor, together with age at presentation and tumor size, while histological features have no prognostic significance.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (7)

adult alveolar soft Part sarcoma · adult alveolar soft part sarcoma · adult alveolar soft-Part sarcoma · alveolar soft Part sarcoma · alveolar soft part sarcoma · alveolar soft part sarcoma (disease) · alveolar soft-part 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

    2,885 matched papers (1,699 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    35 matched on ClinicalTrials.gov (11 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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

2,885

2,885 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=5449) is 41.

2,885 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

1,699 in the last 10 years · low confidence

Phrase hits: 2,885 · MeSH hits: 8

Open Europe PMC search

Who's working on it?

1,372

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
    Wang Y10 papers · 2026

    Department of Medical Oncology, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, 310009, China.

    Papers in Europe PMC
  2. 02
    Wang J7 papers · 2026

    Department of Bone and Soft Tissue Tumor, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.

    Papers in Europe PMC
  3. 03
    Zhang Y7 papers · 2026

    Department of Radiology, The First Affiliated Hospital of Kunming Medical University, Kunming, China.

    Papers in Europe PMC
  4. 04
    Hindi N6 papers · 2026

    Medical Oncology Department, Fundacion Jimenez Diaz University Hospital and Hospital General de Villalba, Madrid; Instituto de Investigación Sanitaria-Fundación Jimenez Díaz-UAM (IIS-FJD-UAM), Madrid, Spain. Electronic address: nhindi@atbsarc.org.

    Papers in Europe PMC
  5. 05
    Liu Y6 papers · 2026

    State Key Laboratory of Systems Medicine for Cancer, Shanghai Cancer Institute, Renji Hospital, School of Medicine, Shanghai Jiaotong University Shanghai, China.

    Papers in Europe PMC
  6. 06
    Chen X5 papers · 2025

    Department of Radiology, Shaanxi Provincial People's Hospital, Xi'an, China.

    Papers in Europe PMC
  7. 07
    Li X5 papers · 2025

    Department of Medical Oncology, the First Hospital of China Medical University, Shenyang, Liaoning Province 110001, China.

    Papers in Europe PMC
  8. 08
    Zhang L5 papers · 2026

    State Key Laboratory of Systems Medicine for Cancer, Shanghai Cancer Institute, Renji Hospital, School of Medicine, Shanghai Jiaotong University Shanghai, China.

    Papers in Europe PMC
  9. 09
    Blay JY4 papers · 2026

    Centre Leon Berard & University Claude Bernard Lyon 1, Lyon, France.

    Papers in Europe PMC
  10. 10
    Huang Y4 papers · 2026

    Department of Nuclear Medicine, China National Nuclear Corporation 416 Hospital, The Second Affiliated Hospital of Chengdu Medical College, Chengdu, Sichuan, P. R. China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

35

interventional trials for this specific condition

35 interventional trials matched this specific condition name; 11 currently recruiting in our sample. 649 trials are registered for soft tissue sarcoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

35 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 96.1th percentile).

low confidence · 96.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: soft tissue sarcoma

649

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

Observational and natural-history studies

2 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.

None of the matched observational studies is currently listed as recruiting.

Open the complete matched search on ClinicalTrials.gov

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 2026-07-26

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.

"Alveolar soft tissue sarcoma" OR "Alveolar soft part sarcoma" OR "adult alveolar soft Part sarcoma" OR "adult alveolar soft-Part sarcoma" OR "alveolar soft part sarcoma (disease)" OR "alveolar soft-part sarcoma"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Sarcoma, Alveolar Soft Part

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

"Alveolar soft tissue sarcoma" OR "Alveolar soft part sarcoma" OR "adult alveolar soft Part sarcoma" OR "adult alveolar soft-Part sarcoma" OR "alveolar soft part sarcoma (disease)" OR "alveolar soft-part sarcoma" OR "Sarcoma, Alveolar Soft Part"

Interventional trials matched via: both, phrase, mesh (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 35 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"soft tissue sarcoma"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: ASPS

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 (2885) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T08:12:49.401Z