ORPHA:263413
Angiosarcoma
Publications
40,003
Trials
59
Interventional, condition-specific
Researchers
1,126
Distinct authors in sample
Gene link
—
Readiness
2/6
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.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0016982
- MeSH:D006394
- UMLS:C0018923
- NCIT:C3088
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.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
40,003 matched papers (19,979 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- 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)
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?
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=5449) is 41.
40,003 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).
19,979 in the last 10 years · low confidence
Phrase hits: 40,003 · MeSH hits: 255
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.
- 01Zhang Y4 papers · 2026
Department of Ultrasound, Longhua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Papers in Europe PMC - 02Kobayashi Y3 papers · 2026
Department of Obstetrics and Gynecology, Institute of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Papers in Europe PMC - 03Liu J3 papers · 2026
Surgery Department of Jingning County People's Hospital, Jingning She Autonomous County, Lishui, China.
Papers in Europe PMC - 04Masuzawa M3 papers · 2026
Department of Dermatology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
Papers in Europe PMC - 05Nakano E3 papers · 2026
Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.
Papers in Europe PMC - 06Namikawa K3 papers · 2026
Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.
Papers in Europe PMC - 07Uchi H3 papers · 2026
Department of Dermato-Oncology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan.
Papers in Europe PMC - 08Wang Y3 papers · 2026
Department of Thoracic Surgery, Weifang No.2 People's Hospital, Weifang, Shandong, China.
Papers in Europe PMC - 09Yoshino K3 papers · 2026
Department of Dermatologic Oncology, The Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan.
Papers in Europe PMC - 10Zhang 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 27 July 2026
59 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 97.4th percentile).
low confidence · 97.4th 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.
- NCT05961761·RECRUITING·Propranolol and Pembrolizumab in Advanced Soft Tissue Sarcoma Patients
Conditions: Soft Tissue Sarcoma Adult · Angiosarcoma · Undifferentiated Pleomorphic Sarcoma·Matched via name phrase
- NCT04859465·RECRUITING·Albumin-bound Paclitaxel Combined With Liposomal Doxorubicin in the Treatment of Advanced or Unresectable Angiosarcoma
Conditions: Angiosarcoma Metastatic·Matched via name phrase
- NCT07007273·RECRUITING·Pembrolizumab for Advanced Cutaneous Sarcomas Not Including Angiosarcoma
Conditions: Cutaneous Sarcoma·Matched via name phrase
- NCT06849986·RECRUITING·IO Combined With AI as First-line Treatment for Patients With Soft Tissue Sarcoma(TAIS)
Conditions: Soft Tissue Sarcomas · Angiosarcoma · Fibrosarcoma · Leiomyosarcoma·Matched via name phrase
- NCT06239272·RECRUITING·NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
Conditions: Adipocytic Neoplasm · Liposarcoma · Atypical Fibroxanthoma · Angiomatoid Fibrous Histiocytoma·Matched via name phrase
- NCT04055220·RECRUITING·Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas
Conditions: Bone Sarcoma · Osteosarcoma · Ewing Sarcoma · Chondrosarcoma·Matched via name phrase
- NCT06638931·RECRUITING·Agnostic Therapy in Rare Solid Tumors
Conditions: Urachal Cancer · Parathyroid Carcinoma · Fibrolamellar Carcinoma · Angiosarcoma·Matched via name phrase
- NCT06673628·RECRUITING·Pembrolizumab Plus Lenvatinib in Unresectable Cutaneous Angiosarcoma Patients
Conditions: Angiosarcoma of Skin · Angiosarcoma Metastatic·Matched via name phrase
- NCT06526897·NOT YET RECRUITING·Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma
Conditions: Adult Pleomorphic Rhabdomyosarcoma · AJCC Grade 2 Sarcoma · AJCC Grade 3 Sarcoma · Alveolar Soft Part Sarcoma·Matched via name phrase
- NCT05607095·RECRUITING·A Study of LN-144 or LN-145 in People With Advanced Uveal Melanoma, Undifferentiated Pleomorphic Sarcoma, Dedifferentiated Liposarcoma, or Angiosarcoma
Conditions: Uveal Melanoma · Melanoma · Metastatic Uveal Melanoma · Metastatic Melanoma·Matched via name phrase
- NCT06440005·RECRUITING·A Study to Evaluate Safety, Tolerability and Preliminary Activity of AGX101 in Participants With Advanced Solid Tumors
Conditions: Cancer · Advanced Cancer · Locally Advanced Carcinoma · Metastatic Solid Tumor·Matched via name phrase
- NCT05859074·RECRUITING·A Study of MQ710 With and Without Pembrolizumab in People With Solid Tumor Cancer
Conditions: Cutaneous Squamous Cell Carcinoma · SCC - Squamous Cell Carcinoma · Basal Cell Carcinoma · BCC·Matched via name phrase
- NCT01042379·RECRUITING·I-SPY TRIAL: Neoadjuvant and Personalized Adaptive Novel Agents to Treat Breast Cancer
Conditions: Breast Neoplasms · Breast Cancer · Breast Tumors · Angiosarcoma·Matched via name phrase
- NCT07655570·NOT YET RECRUITING·Pilot Study of Ivonescimab in Advanced/Metastatic Cutaneous Angiosarcoma
Conditions: Cutaneous Angiosarcoma·Matched via name phrase
- NCT06277154·RECRUITING·MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma
Conditions: Leiomyosarcoma · Liposarcoma · Synovial Sarcoma · Angiosarcoma·Matched via name phrase
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
- NCT06715579·RECRUITING·Cardiac Angiosarcoma International Registry
Conditions: Cardiac Angiosarcoma·Matched via name phrase
- NCT07432932·RECRUITING·Precision Medicine Approaches for Neoadjuvant Therapy in High-risk Sarcoma Patients
Conditions: Sarcoma · Sarcoma, Leiomyo-, Adult · Sarcoma, Synovial, Adult · Soft Tissue Sarcoma (STS)·Matched via name phrase
- NCT05515068·NOT YET RECRUITING·Registry For Children, Adolescents And Adults With Osteosarcoma And Biologically Related Bone Sarcomas
Conditions: Osteosarcoma · Bone Tumor · Bone Sarcoma · High Grade Sarcoma·Matched via name phrase
- NCT06375941·RECRUITING·Prospective Observational Study of Localized Angiosarcoma of Any Site: ProStars
Conditions: Angiosarcoma·Matched via name phrase
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 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"
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
