Epithelioid Hemangioendothelioma (EHE) is a rare cancer that belongs to the group of cancers called sarcomas. EHE is a vascular sarcoma. This cancer can occur anywhere in the body with the most common sites being the liver, lungs, and bone. Symptoms depend on where the cancer develops in the body.
Last updated 04/30/2025
Clinical
Disease Class
Neoplastic diseases
Body Systems
Cardiovascular / Circulatory
Digestive
Endocrine
Hematopoietic / Lymphatic / Immune
Integumentary / Exocrine
Muscular / Skeletal
Renal / Urinary / Excretory
Reproductive
Respiratory
Organs
Blood
Brain
Connective tissue / joints
Ears
Heart
Intestines
Kidneys
Liver
Lungs
Lymph fluid, nodes, ducts, vessels
Muscles
Nose
Pancreas
Skin
Throat/pharynx
Known Genetic Link
No, the disease is not known to have a genetic basis
causative_genes
None specified / unknown
contributory_genes
None specified / unknown
Type of Inheritance
Not specified / unknown
Newborn Screening
No
Disease Mechanism(s)
Altered DNA repair mechanism
Pathogenic mutation
mTOR pathways dysregulation
Age of Onset
Adolescence (12-17)
Adulthood (age 18-64)
Early childhood (age 1+-5)
Elderly (age 65+)
Infancy (age 0-1)
Middle childhood (6-11)
Average Age at Diagnosis
Adulthood (age 18-64)
Life Expectancy
Adulthood (age 18-64)
Affected Sex(es)
Female
Male
National Prevalence
101-1000
Global Prevalence
1001-10000
National Incidence
Less than 10
Global Incidence
Less than 10
Symptoms / Phenotypes
abdominal swelling
ascites
balance problems
bone break / fracture
breathing difficulties
cancer / carcinoma / tumor / malignancy, unspecified
fever
pain, abdominal
pleural effusion
skin redness and/or swelling
vomiting / nausea
weight loss
Biomarkers
None
Existing Therapies
Off-Label Drug Use
Organizational & Research
Cell Lines
Cancer/tumor cells
Cell Lines, Institution
University of Iowa
Cell Lines, Involvement
Funded
Own
Cell Lines, share
No
Disease Model
Mouse
Disease Model, Involvement
Funded
Disease Model, share
No
Clinical Trial Role
Outcome measures, development
Recruitment and outreach, patients
Recruitment and outreach, trial sites/physicians
Biobank, Institution
Cleveland Clinic BioRepository (CC-BioR)
Biobank, Involvement
Funded
Own
Center of Excellence, Institution
Memorial Sloan Kettering Cancer Center
Center of Excellence, Involvement
Consulted
Funded
Registry
Yes, we have a registry that we created
Data Collected, Registry
Longitudinal natural history data
Medication usage
Other
Patient contact info
Patient-reported data
Data Entered by, Registry
Patients
Platform, Registry
IAMRARE
Natural History Study
No, we do not have a natural history study, but we plan to create or collaborate on one
FDA Patient Listening Session
No
FDA Patient-Focused Drug Development (PFDD) Program