ORPHA:458758
Composite hemangioendothelioma
Also known as: Polymorphous hemangioendothelioma
Publications
150
66.2th percentile
Trials
0
Interventional, condition-specific
Researchers
723
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare vascular tumor characterized by a poorly circumscribed, infiltrative nodular lesion with vascular differentiation, centered in the dermis and subcutis. The tumor is composed of histologically benign, intermediate, and malignant components. Typical is an admixture of different components which include epithelioid and retiform hemangioendothelioma, spindle cell hemangioma, angiosarcoma-like areas, and benign vascular lesions. Predilection sites are the distal extremities. Many patients have a history of lymphedema. Local recurrence is frequent, while metastasis is rare.
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:0018712
- UMLS:C1304513
- NCIT:C45475
Additional Mondo synonyms (1)
composite hemangioendothelioma
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
No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
150 matched papers (99 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 trialPartial
None under the specific name; 24 for broader category hemangioendothelioma
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?
150
150 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
150 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).
99 in the last 10 years · high confidence · 66.2th percentile (publications denominator)
Phrase hits: 150 · MeSH hits: 0
Who's working on it?
723
Distinct author names in 150 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01Antonescu CR10 papers · 2026
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Papers in Europe PMC - 02Dermawan JK5 papers · 2024
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Papers in Europe PMC - 03Fletcher CD4 papers · 2000
Department of Pathology, Harvard Medical School, Boston, MA, USA.
Papers in Europe PMC - 04Folpe AL4 papers · 2024
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Papers in Europe PMC - 05Agaimy A3 papers · 2025
Institute of Pathology, Erlangen University Hospital, Comprehensive Cancer Center, European Metropolitan Area Erlangen-Nuremberg, Friedrich Alexander University of Erlangen-Nuremberg, Erlangen, Germany.
Papers in Europe PMC - 06Dickson BC3 papers · 2023
Department of Pathology & Laboratory Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada.
Papers in Europe PMC - 07Kutzner H3 papers · 2013Papers in Europe PMC
- 08Linos K3 papers · 2025
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
Papers in Europe PMC - 09
- 10Rubin BP3 papers · 2017
Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 24 trials are registered for hemangioendothelioma, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
high confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
24 interventional trials matched hemangioendothelioma, the broader category — listed below. Those studies are not counted in the condition-specific total.
Broader category: hemangioendothelioma
24
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
- NCT07104331·RECRUITING·SARC046: A Phase II Trial of Nab-Sirolimus in Patients With Progressing or Symptomatic Epithelioid Hemangioendothelioma
Conditions: Epithelioid Hemangioendothelioma (EHE)·Matched via name phrase
- NCT07684287·NOT YET RECRUITING·A Phase 2 Study of Sirolimus for Injection (Albumin-bound) in Patients With Progressive or Symptomatic Epithelioid Hemangioendothelioma
Conditions: Epithelioid Hemangioendothelioma·Matched via name phrase
- NCT07477548·NOT YET RECRUITING·A Study to Evaluate the Efficacy and Safety of Everolimus in Patients With Teratment-refractory Vascular Anomalies
Conditions: Vascular Malformations · Arteriovenous Malformations · Venous Malformation · Lymphangioma·Matched via name phrase
- NCT07131644·NOT YET RECRUITING·Sirolimus Discontinuation Strategies in Kaposiform Hemangioendothelioma
Conditions: Kaposiform Hemangioendothelioma·Matched via name phrase
- NCT06452160·RECRUITING·A Study of BGC515 Capsules in Subjects With Advanced Solid Tumors
Conditions: Mesothelioma · Epithelioid Hemangioendothelioma(EHE) · Solid Tumor·Matched via name phrase
- NCT03967834·RECRUITING·Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group
Conditions: Soft Tissue Sarcoma · Clear Cell Sarcoma · Epithelioid Sarcoma · Perivascular Epithelioid Cell Neoplasms·Matched via name phrase
- NCT07656909·RECRUITING·Low- vs High-Dose Sirolimus With Prednisolone for KHE and KMP
Conditions: Kaposiform Hemangioendothelioma (KHE) · Kasabach Merritt Phenomenon·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.
"Composite hemangioendothelioma" OR "Polymorphous hemangioendothelioma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Composite hemangioendothelioma" OR "Polymorphous hemangioendothelioma"
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"hemangioendothelioma"
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T16:52:09.664Z