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epithelioid hemangioendothelioma - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

epithelioid hemangioendothelioma

MONDO:0015523

Also known as:epithelioid angioendotheliomaepithelioid angiosarcomamalignant epithelioid hemangioendothelioma

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Overview

A low-grade malignant blood vessel neoplasm. It is characterized by the presence of epithelioid endothelial cells. The neoplastic cells are arranged in cords and nests, which are embedded in a myxoid to hyalinized stroma.

TreatmentsNo approved treatments · 1 in pipeline

Clinical Trials8 registered · 6 recruiting

Diagnosis

Biomarker and diagnostic research for epithelioid hemangioendothelioma has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for epithelioid hemangioendothelioma. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for epithelioid hemangioendothelioma, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for epithelioid hemangioendothelioma. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

8 clinical trials registered, 6 recruiting. Interventions under study include drug therapy, other interventions, and procedural interventions. Pipeline includes 3 PHASE2, 2 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT03331250](https://clinicaltrials.gov/study/NCT03331250)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:47 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:157791

GARD

Genetic and Rare Diseases Info Center

GARD:20014

Questions for Your Care Team

→Are any of the 8 active clinical trials a good fit for my situation?
→What is the current development status of N-methyl-3-(1-methyl-1H-imidazol-4-yl)-4-((4-(trifluoromethyl)benzyl)amino)benzenesulfonamide, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Research Type	Count	% of Total
Patient case studies	171	64%
Research summaries	30	11%
Clinical study results	17	6%
Laboratory research	13	5%
Disease patterns and progression	11	4%
Testing and diagnosis research	10	4%
Other research	8	3%
New treatment approaches	6	2%

epithelioid hemangioendothelioma

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

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