Epithelioid hemangioendothelioma

Summary

Epithelioid hemangioendothelioma (MONDO:0015523) is a disease and 7 clinical trials. Top therapeutic interventions include eribulin and trametinib. A subtype of vascular cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide)
• Clinical trials: 7

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	epithelioid hemangioendothelioma
Mondo ID	MONDO:0015523
MeSH	D018323
Orphanet	157791
DOID	DOID:0080190
NCIT	C3800
SNOMED CT	403981003, 54124005
UMLS	C0334538
MedGen	569676
GARD	0020014
NORD	124536
Is cancer (heuristic)	no

Also known as: epithelioid angioendothelioma · epithelioid angiosarcoma · epithelioid hemangioendothelioma · malignant epithelioid hemangioendothelioma

Data availability: 3 cell lines.

Disease family

This is a subtype of vascular cancer. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › cardiovascular cancer › vascular cancer › epithelioid hemangioendothelioma

Related subtypes (6): choroid plexus cancer, malignant jugulotympanic paraganglioma, adrenal cortex carcinoma, choroid cancer, angiosarcoma, great vessel cancer

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Eribulin, Trametinib.

Clinical trials & evidence

Clinical trials

Clinical trials: 7.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	3
PHASE1	2
Not specified	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03331250	PHASE2	ACTIVE_NOT_RECRUITING	Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE)
NCT07104331	PHASE2	RECRUITING	SARC046: A Phase II Trial of Nab-Sirolimus in Patients With Progressing or Symptomatic Epithelioid Hemangioendothelioma
NCT03148275	PHASE2	COMPLETED	Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery
NCT06452160	PHASE1	RECRUITING	A Study of BGC515 Capsules in Subjects With Advanced Solid Tumors
NCT05228015	PHASE1	TERMINATED	Oral TEAD Inhibitor Targeting the Hippo Pathway in Subjects with Advanced Solid Tumors
NCT03967834	Not specified	RECRUITING	Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group
NCT06408441	Not specified	RECRUITING	The Epithelioid Hemangioendothelioma Registry of the European Reference Network on Rare Adult Solid Cancers (EURACAN)

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ERIBULIN	4	1
TRAMETINIB	4	1
CHEMBL5433950	0	1