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ORPHA:263413

Angiosarcoma

low confidenceDisorder

Publications

40,003

Trials

59

Interventional, condition-specific

Researchers

1,126

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare vascular tumor characterized by a malignant space-occupying lesion composed of cells variably recapitulating features of normal endothelium. It mostly develops as a cutaneous tumor and is much less frequently located in the deep soft tissue. Clinical presentation is an enlarging mass, sometimes with symptoms like coagulopathy, anemia, persistent hematoma, or bruisability. Some tumors are associated with pre-existing conditions, e. g. Klippel-Trenaunay syndrome, Maffucci syndrome, or following radiation, among others. Older age, retroperitoneal location, large size, and high mitotic activity are predictors for poor outcome.

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 (9)

angiosarcoma · angiosarcoma (disease) · blood vessel sarcoma · hemangiosarcoma · hemangiosarcoma, malignant · malignant angioendothelioma · malignant hemangioendothelioma · sarcoma of blood vessel · vascular 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.”

4/6 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

    40,003 matched papers (19,979 in last 10 years) Source

  3. Phenotype characterisedPresent

    27 HPO annotations (e.g. Abnormality of the spleen; Immunodeficiency; Lymphoproliferative disorder) Source

  4. Animal modelPresent

    3 genotype models (Danio rerio, Mus musculus) Source

  5. Orphan designationNot found

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

  6. Interventional trialPresent

    59 matched on ClinicalTrials.gov (15 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)

27

Associated phenotypes · MONDO:0016982

  • Abnormality of the spleen
  • Immunodeficiency
  • Lymphoproliferative disorder
  • Abnormality of the liver
  • Recurrent herpes

Showing 5 of 27 — open Monarch for the full list.

Animal models (Monarch / Alliance)

3

Model associations linked to this Mondo ID

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

38

Drugs / clinical candidates · MONDO_0016982

CTD chemicals (MyDisease.info)

33 associated chemicals · 458 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Doxorubicin · therapeutic
  • Paclitaxel · therapeutic
  • peginterferon alfa-2b · therapeutic
  • 1-(2-hydroxyethyl)-1-nitrosourea · marker/mechanism
  • 1,2-Dimethylhydrazine · marker/mechanism
  • 1,3-butadiene · marker/mechanism
  • 2-Acetylaminofluorene · marker/mechanism
  • 2-nitrotoluene · marker/mechanism
  • 4-chloroaniline · marker/mechanism
  • 4-nitroaniline · marker/mechanism
  • Aminopyrine · marker/mechanism
  • ANFT · marker/mechanism

Pathways: Inositol phosphate metabolism; Metabolic pathways; EGFR tyrosine kinase inhibitor resistance; Endocrine resistance; Platinum drug resistance; MAPK signaling pathway; ErbB signaling pathway; Ras signaling pathway

MyDisease.info · MONDO:0016982

Literature

Is anyone studying this?

40,003

40,003 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=3967) is 59.

40,003 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).

19,979 in the last 10 years · low confidence

Phrase hits: 40,003 · MeSH hits: 255

Open Europe PMC search

Who's working on it?

1,126

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 Y4 papers · 2026

    Department of Ultrasound, Longhua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China.

    Papers in Europe PMC
  2. 02
    Kobayashi Y3 papers · 2026

    Department of Obstetrics and Gynecology, Institute of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.

    Papers in Europe PMC
  3. 03
    Liu J3 papers · 2026

    Surgery Department of Jingning County People's Hospital, Jingning She Autonomous County, Lishui, China.

    Papers in Europe PMC
  4. 04
    Masuzawa M3 papers · 2026

    Department of Dermatology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.

    Papers in Europe PMC
  5. 05
    Nakano E3 papers · 2026

    Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.

    Papers in Europe PMC
  6. 06
    Namikawa K3 papers · 2026

    Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.

    Papers in Europe PMC
  7. 07
    Uchi H3 papers · 2026

    Department of Dermato-Oncology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan.

    Papers in Europe PMC
  8. 08
    Wang Y3 papers · 2026

    Department of Thoracic Surgery, Weifang No.2 People's Hospital, Weifang, Shandong, China.

    Papers in Europe PMC
  9. 09
    Yoshino K3 papers · 2026

    Department of Dermatologic Oncology, The Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan.

    Papers in Europe PMC
  10. 10
    Zhang H3 papers · 2026

    Department of Ultrasound, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

59

interventional trials for this specific condition

59 interventional trials matched this specific condition name; 15 currently recruiting in our sample.

Data as of 11 September 2026

59 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 97.5th percentile).

low confidence · 97.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

7 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

Open the complete matched search on ClinicalTrials.gov

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 9 · after dedupe 9 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 9 · 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 (9)

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 Angiosarcoma — 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.

"Angiosarcoma" OR "angiosarcoma (disease)" OR "blood vessel sarcoma" OR "hemangiosarcoma" OR "hemangiosarcoma, malignant" OR "malignant angioendothelioma" OR "malignant hemangioendothelioma" OR "sarcoma of blood vessel" OR "sarcoma of the blood vessel" OR "vascular sarcoma"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Hemangiosarcoma

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

"Angiosarcoma" OR "angiosarcoma (disease)" OR "blood vessel sarcoma" OR "hemangiosarcoma" OR "hemangiosarcoma, malignant" OR "malignant angioendothelioma" OR "malignant hemangioendothelioma" OR "sarcoma of blood vessel" OR "sarcoma of the blood vessel" OR "vascular 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: 59 interventional · 7 observational · 0 expanded access. Only interventional studies enter the trial headline.

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

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is short or not clearly distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (40003) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-27T11:20:25.067Z