Epithelioid sarcoma (ES) is a rare cancerous tumor that most often occurs in the soft tissue of the fingers, hands and forearms of young adults. It can also occur elsewhere in the body. ES usually begins as a painless, firm growth or bump that may be accompanied by an open wound (ulceration) in the skin covering the growth. This type of tumor often comes back after treatment or spreads to other parts the body (metastasis). The cause of epithelioid sarcoma is unknown. It is diagnosed by a clinical examination and by testing a small sample of the tumor (biopsy) in a laboratory. Epithelioid sarcoma is treated with surgery to remove all the cancercells (wide local excision) and sometimes with radiation therapy. Chemotherapy may also be used if the cancer has spread to other parts of the body.

Some of the symptoms may include:

• Painless growth on the extremities
• Break on the skin (ulceration)

Often people with epithelioid sarcoma have few or no other symptoms. ES typically occurs in young adults, but it can occur at any age. Males are more often affected than females. ES is an aggressive tumor and often comes back after treatment or spreads to other parts of the body. The long term outcome depends on how aggressive the tumor is.

The exact cause of epithelioid sarcoma is unknown. About 30% of people have had a previous trauma at the site where the tumor grows, but it is unclear if this has any significance.

Epithelioid sarcoma is diagnosed based on a clinical examination and an examination of a piece of the tumor in the laboratory (biopsy).

Specialists who may be involved in the care of someone with ES include:

• Oncologist
• Radiation oncologist
• Surgeon
• Pathologist