ULTRA-RARE CANCERVascular TumorWHO 5th Edition Classification

Epithelioid Hemangioendothelioma (EHE)

Ultra-Rare Malignancy (< 1 case per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A vascular tumour of intermediate malignancy arising from the cells that line blood vessels. Its behaviour is unusually variable - some patients remain stable without treatment for years, while others have rapidly progressive disease. Liver, lung and bone are the commonest sites.

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Who It Affects

Adults across a wide age range; slight female predominance

Annual Incidence

Approximately 1 per million per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

Often none - frequently found incidentally on imaging
Pain at the affected site, especially bone lesions
Cough, breathlessness or pleuritic pain with lung involvement
Right upper abdominal discomfort with liver involvement
Fatigue, weight loss and fever in aggressive disease

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

WWTR1-CAMTA1 fusion (about 90%)YAP1-TFE3 fusion (variant)

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

Active surveillance for stable, asymptomatic disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Surgical resection of localised lesions

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

Liver transplantation in selected hepatic-only disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

mTOR inhibition (sirolimus)

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Antiangiogenic therapy or interferon in progressive disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

Highly variable. Many patients live for many years with stable disease. Serosal effusions, systemic symptoms and high mitotic count identify the aggressive subgroup with markedly shorter survival.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

The EHE Rare Cancer Charity and international consortia are running trials of TAZ/YAP-TEAD inhibitors, which target the fusion oncoprotein directly; trametinib and sirolimus have prospective data.

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Expert Clinicians

Verified Specialists for Epithelioid Hemangioendothelioma

Browse All Oncologists

Dr. Vinod Ravi

Sarcoma Medical Oncologist

20+ yrs exp

MD Anderson

Houston, USA

Sarcoma OncologyVascular SarcomasEHE
Vetted DirectoryConsult Specialist

Dr. Richard Quek

Senior Consultant Medical Oncologist

16+ yrs exp

Sarcoma Consortium

Singapore, Singapore

Sarcoma OncologyEHERare Sarcomas
Vetted DirectoryConsult Specialist

Prof. Jean-Yves Blay

Oncologist, Director

25+ yrs exp

Centre Léon Bérard

Lyon, France

Rare SarcomasEHEAngiosarcomas
Vetted DirectoryConsult Specialist

Dr. Sameer Rastogi

Sarcoma Medical Oncologist

15+ yrs exp

AIIMS, New Delhi

New Delhi, India

Sarcoma OncologyRare Vascular TumorsEHE
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

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Independent Tumor Board Review

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