Epithelioid Hemangioendothelioma

Also known as: EHE

What is epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma, or EHE, is a rare vascular tumor of young adults (though can occur in young children) that starts in the cells lining the blood vessels of soft-tissue, bone and body organs- commonly the liver, lungs and bone. They vary in how they grow. In many children the tumor is non-cancerous (benign) and grows very slowly (and may even disappear on its own); in others, it can act like a cancer, growing rapidly, and spread to other parts of the body, causing a number of complications.

What causes epithelioid hemangioendothelioma?
The cause is unknown, however some genetic mutations have recently been seen in some tumors which may be related to its development.

What are the symptoms of epithelioid hemangioendothelioma?
As the tumor can arise anywhere in the body, symptoms will depend on its location, size and spread. If the tumors are benign or slow growing, they may not cause any symptoms. In more severe instances, common symptoms include weight loss, pain and swelling in the tumor area; other symptoms will depend on the specific organs involved.

What are epithelioid hemangioendothelioma care options?
Depending on the rate of tumor growth, its size and whether any complications are present, some surgeons may elect to watch and monitor the tumor; others will undertake surgery to remove as much of the tumor as possible. Other treatments include chemotherapy, radiation therapy and/or other special targeted medications that may be useful in shrinking the tumors and preventing their growth or spread.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 5/23/2018 11:12:29 AM

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