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ORPHA:569164

Angiomatoid fibrous histiocytoma

medium confidenceDisorder

Also known as: AFH

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

917

90.5th percentile

Trials

4

Interventional, condition-specific

Researchers

1,241

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare soft tissue tumor characterized by a slow-growing, usually painless, subcutaneous nodule, predominantly located in the extremities, less frequently the trunk or head and neck region. Histopathologically, the lesion is well-circumscribed, lobulated, and composed of epitheloid, ovoid, or spindle cells arranged in a nodular and often syncytial pattern, with pseudoangiomatoid spaces and a peripheral fibrous pseudocapsule with a prominent lymphoplasmacytic cuff. The tumor is most common in the first two decades of life and usually follows an indolent course, although local recurrence may occur, while metastasis is rare.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

angiomatoid fibrous histiocytoma · angiomatoid malignant fibrous histiocytoma · histiocytoma, Angiomatoid fibrous · histiocytoma, angiomatoid fibrous, somatic

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

    917 matched papers (524 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

    4 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)

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?

917

917 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.

917 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).

524 in the last 10 years · medium confidence · 90.5th percentile (publications denominator)

Phrase hits: 917 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,241

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
    Yoshida A8 papers · 2025

    Department of Diagnostic Pathology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

    Papers in Europe PMC
  2. 02
    Agaimy A6 papers · 2025

    Institute of Pathology, Friedrich-Alexander University Erlangen-Nürnberg (FAU), University Hospital Erlangen (UKER), Erlangen, Germany. abbas.agaimy@uk-erlangen.de.

    Papers in Europe PMC
  3. 03
    Wang Y5 papers · 2026

    School of Medicine, International Peace Maternity and Child Health Hospital, Shanghai Jiao Tong University, Shanghai Key Laboratory of Embryo Original Diseases, Shanghai, 200030, China. wyanlin@163.com.

    Papers in Europe PMC
  4. 04
    Antonescu CR4 papers · 2026

    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA. antonesc@mskcc.org.

    Papers in Europe PMC
  5. 05
    Dermawan JK4 papers · 2026

    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

    Papers in Europe PMC
  6. 06
    Michal M4 papers · 2026

    Biopticka Laborator Ltd., Pilsen, Czech Republic.

    Papers in Europe PMC
  7. 07
    Stoehr R4 papers · 2025

    Institute of Pathology, Friedrich-Alexander University Erlangen-Nürnberg (FAU), University Hospital Erlangen (UKER), Erlangen, Germany.

    Papers in Europe PMC
  8. 08
    Benzerdjeb N3 papers · 2026

    Department of Pathology, Institut de Pathologie Multisite, CHU Sud, 165 Chemin du Grand-Revoyet, Hospices Civils de Lyon, 69310, Pierre Bénite, France. nazim.benzerdjeb@chu-lyon.fr.

    Papers in Europe PMC
  9. 09
    Chen J3 papers · 2026

    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha.

    Papers in Europe PMC
  10. 10
    Chen X3 papers · 2026

    Department of Pathology, Ruijin Hospital of Shanghai Jiaotong University, Shanghai, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

4

interventional trials for this specific condition

4 interventional trials matched this specific condition name; 1 currently recruiting in our sample. 20 trials are registered for histiocytoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

4 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 86.7th percentile).

medium confidence · 86.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: histiocytoma

20

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

1 observational study matches 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.

"Angiomatoid fibrous histiocytoma" OR "angiomatoid malignant fibrous histiocytoma" OR "histiocytoma, Angiomatoid fibrous" OR "histiocytoma, angiomatoid fibrous, somatic"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Histiocytoma, Angiomatoid Fibrous

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

"Angiomatoid fibrous histiocytoma" OR "angiomatoid malignant fibrous histiocytoma" OR "histiocytoma, Angiomatoid fibrous" OR "histiocytoma, angiomatoid fibrous, somatic"

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: 4 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"histiocytoma"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: AFH

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

Ingested 2026-07-27T18:30:00.001Z