Epithelioid hemangioendothelioma (EHE)

Overview

An epithelioid hemangioendothelioma (EHE) is a rare cancer called a vascular sarcoma. It typically starts in cells called endothelial cells. These cells line blood vessels. They can form almost anywhere in the body. Common areas include the liver, lungs, soft tissue and bones.

EHE is part of the sarcoma family of cancers. It is not the same as an angiosarcoma, even though the two cancers can look alike under a microscope.

EHE can happen at any age. It most often is diagnosed in middle-aged adults. It is very rare in children.

EHE can act differently from one person to another. Some EHE tumors may grow slowly for years. Other EHE tumors may grow quickly. Treatment depends on where the EHE is located, whether it causes symptoms and whether it has spread to other parts of the body.

Symptoms

EHE does not always cause symptoms. Sometimes EHE tumors are found on scans done for another reason.

EHE symptoms can vary based on where the tumor is in the body. Symptoms in these areas may include:

• Lungs: Cough, chest pain, or shortness of breath.
• Liver: Large tumors may cause pain or discomfort in the belly area.
• Soft tissues: A lump or pain.
• Bones: Swelling, pain or broken bone.

When to see a doctor

Make an appointment with a healthcare professional if you have symptoms that worry you or are not going away.

Causes

The cause of EHE is not known. Researchers found certain gene changes in EHE tumor cells, but it’s not clear what causes these gene changes to happen.

Risk factors

The risk factors for EHE are not known.

Complications

EHE complications may include:

• Tumor spread, also called metastasis, within an organ or to other parts of the body.
• Fluid buildup around the lungs or in the belly.

Prevention

Researchers do not know what causes EHE. Factors that may increase a person's risk of developing EHE are not known.