Epithelioid Sarcoma

Also known as: soft tissue sarcoma

What is epithelioid sarcoma?

Epithelioid sarcoma is a rare form of cancer that most commonly affects the arms and hands but can also be found in the legs, feet or main part of the body. It’s a slow-growing cancer that usually responds well to treatment.

What causes epithelioid sarcoma?

Like most cancers, the root cause of epithelioid sarcoma is due to changes in a cell’s DNA. What specifically causes this change to occur isn’t clear. Some people with certain family cancer syndromes may be at a greater risk of developing it.

What are the symptoms of epithelioid sarcoma?

In many cases, epithelioid sarcoma doesn’t cause any noticeable symptoms. As the tumor grows, it may cause symptoms such as an obvious or growing lump in the area, pain and swelling or open wounds.

How can epithelioid sarcoma affect children?

Epithelioid sarcoma is rare and exhibits similarly in both children and adults. The cancer occurs most commonly in early adulthood.

What are epithelioid sarcoma treatments?

Surgery, chemotherapy, radiation therapy or some combination of the three treatments is the standard treatment method for epithelioid sarcoma.

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Reviewed by: Maggie Fader, MD

This page was last updated on: July 29, 2025 04:27 PM