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Epithelioid Hemangioendothelioma Treatment

Epithelioid hemangioendothelioma (EHE) is a rare type of vascular tumor that affects the cells that line the inside of blood vessels (the vascular system). These cells are called epithelial cells. EHE tumors can appear in blood vessels anywhere in the body. They usually affect the soft tissues, liver, lungs, and bones. The cause of EHE is not known.

Vascular tumors like EHE are uncommon. They represent about 2% of soft tissue tumors in children and teens.

These tumors are cancerous. How fast they grow and how they affect the person vary widely between patients. Some people can live with these tumors for years with only minor symptoms that affect daily life. Sometimes the tumors even go away on their own. Other tumors can grow and spread quickly.

Epithelioid hemangioendothelioma symptoms

EHE symptoms often depend on where the tumor is located. The most common symptoms include:

  • Weight loss
  • Pain and swelling in the tumor area, such as abdominal (belly) pain
  • Mass in the tumor area
  • Skin bumps that may be red or blue
  • Swollen organ caused by the tumor, such as the liver or spleen
  • Problems moving or walking if the tumor occurs in the spine

Epithelioid hemangioendothelioma treatment

EHE treatment depends on the location of the tumor and how severe it is.

A common treatment for EHE is surgery. During surgery, the surgeon removes the tumor and a small amount of tissue around the tumor. The healthy tissue that is removed is called a margin. Removing a margin of tissue makes sure the surgeon removes the whole tumor.

The care team may also use radiation, chemotherapy, or targeted therapies to treat EHE.

A person with EHE in the liver may receive a liver transplant.

In some cases, if the tumor spreads slowly, your child’s care team may take a wait-and-see approach. They monitor the tumor’s growth. This prevents your child from having treatments that are not needed or that may be harmful.

Epithelioid hemangioendothelioma prognosis

Survival rates for EHE are not well known. Rates depend on the tumor location and type of treatment:

  • Liver EHE: A 2006 study of 434 people with this type of EHE found 5-year survival rates of 55% in people who had liver transplants and 30% in people who had chemotherapy or radiation. Two studies since then have shown even higher rates of survival for people who got liver transplants.
  • Soft-tissue EHE. This type of EHE may have a survival rate of 13–18%.

Epithelioid hemangioendothelioma care at St. Jude

St. Jude provides the highest quality of care for patients with EHE:

  • We have a dedicated team of specialists to meet the needs of children with EHE, including:
    • Surgeons, doctors, and nurses who treat this cancer
    • Doctors who specialize in radiation therapy and pathology (making a diagnosis by looking at tumor tissue under the microscope)
    • Experts in diagnostic imaging and nuclear medicine
    • Genetic counselors
    • Clinical dietitians
    • Child life specialists
    • Psychologists
    • Researchers and scientists
  • The care team works closely with researchers to bring new treatments from the lab to the clinic.

More reasons to choose St. Jude for care include:

  • We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report. 
  • At St. Jude, we have created an environment where children can be children and families can be together.  
  • St. Jude patients and families never pay for services authorized by St. Jude. 
  • We lead more clinical trials for childhood cancer than any other hospital in the U.S.  
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer. 
  • The nurse-to-patient ratio at St. Jude is unmatched. It is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit. 
  • Patients may be able to get expert, compassionate care and treatment closer to their homes through the St. Jude Affiliate Program. 

Clinical trials for EHE

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults for EHE.

Learn more about clinical research at St. Jude.

Open clinical trials

NRSTS2021: A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy, and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma

Study goal:

The purpose of this clinical trial is to find better treatments for NRSTS patients who are intermediate- and high-risk. Our goal is to lower the risk of the tumor coming back after treatment, while using treatments with fewer risks of long-term side effects.


Up to 30 years old.

A statue of children running and holding hands

Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact the Physician / Patient Referral Office

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Fax: 901-595-4011 | Email: | 24-hour pager: 1-800-349-4334

The solid tumor coordinator is dedicated to helping you seek treatment or refer a patient.

Solid Tumor Coordinator


Learn more