Epithelioid Hemangioendothelioma

What is epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones.

These tumors are malignant (cancerous). But they are slow-growing and do not usually spread (metastasize) as quickly as other cancers. Because they are so rare, they can go undetected for a long time before being diagnosed. This gives the tumors time to spread to surrounding tissues.

Epithelioid hemangioendothelioma tumors affect each person differently. Some people can live with these slow-growing tumors for years with only minor disruption to daily life. Sometimes the tumors even go away on their own. Less commonly, the tumors grow and spread quickly.

The cause of epithelioid hemangioendothelioma is not known.

How common is epithelioid hemangioendothelioma?

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

What are the signs and symptoms of epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma can appear anywhere in the body. Its symptoms often depend on the tumor’s location. The most common symptoms include the following:

• Weight loss
• Pain and swelling in the tumor area, such as pain in the abdomen
• Mass in the tumor area
• Skin nodules that may be red or blue
• Enlarged organ affected by the tumor, such as the liver or spleen
• Problems moving or walking, such as if the tumor occurs in the spine

How is epithelioid hemangioendothelioma treated?

Epithelioid hemangioendothelioma treatment varies based on tumor location and grade (how severe it is).

A common treatment is surgery. The surgeon will remove as much surrounding tissue as needed to provide a margin of healthy tissue.

Doctors may also use radiation, chemotherapy or targeted therapies to treat epithelioid hemangioendothelioma.

A person with epithelioid hemangioendothelioma in the liver may receive a liver transplant.

In some cases, if the tumor is spreading slowly, your child’s doctor may take a wait-and-see approach to monitor tumor growth rather than subject your child to the risks and side effects of treatment.

What are the survival rates for epithelioid hemangioendothelioma?

Survival rates for epithelioid hemangioendothelioma are not well known and vary depending on the tumor location and type of treatment:

• Liver epithelioid hemangioendothelioma: A 2006 study of 434 people with this type of epithelioid hemangioendothelioma found five-year survival rates of 55% in people with liver transplants and 30% in people who had chemotherapy or radiation. Two large-scale studies since then have shown even higher rates of survival for people who received liver transplants.
• One estimate places the mortality rate for soft-tissue epithelioid hemangioendotheliomas at 13% to 18%.

Why choose St. Jude for your child’s epithelioid hemangioendothelioma treatment?

• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
• Your child will have access to a close-knit team of specialists that includes: surgeons; doctors and nurses who treat this cancer; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosis (pathologists); experts in diagnostic imaging and nuclear medicine; dietitians; speech therapists; rehabilitation specialists (physical therapists, occupational therapists); child life specialists; psychologists; Quality of Life team members; experts who help manage and minimize the long-term or late effects of treatment; researchers; scientists; and many others.
• The medical team works closely with laboratory researchers to bring new treatments from the laboratory to the clinic.

Keywords:

Primary: epithelioid hemangioendothelioma

Secondary: EHE, HEHE, vascular tumor, vascular cancer, hepatic epithelioid hemangioendothelioma