Epithelioid Sarcoma

Epithelioid sarcoma (ES) is a rare subtype of soft tissue sarcoma. It usually starts as a lump in your forearms, hands, fingers, lower legs or feet — though it can appear in other areas. Eventually, more than one lump may develop, but this isn’t always the case. In some instances, you may also have open wounds (ulcerations) on your skin above the lumps.

Epithelioid sarcoma is slow growing. A person can have this type of cancer for months (or even years) before noticing any symptoms.

Another name for this condition is epithelial sarcoma. There are two types: distal and proximal.

What are the types of epithelioid sarcomas?

• Distal type is the most common form of epithelioid sarcoma. Distal type ES affects your extremities, such as your hands, feet, lower arms and lower legs.
• Proximal type is the rarest form of ES. Proximal type epithelioid sarcoma (PES) affects areas closer to the center of your body. This can include your chest, abdomen, armpits, genitals (penis or vulva) or head and neck. Proximal type epithelioid sarcoma is typically more aggressive than distal type ES. As a result, it’s usually more challenging to treat.

How common is epithelioid sarcoma?

Epithelioid sarcoma is rare. To put it in perspective, soft tissue sarcomas (which include more than 50 subtypes) account for approximately 1% of all adult cancers. Epithelioid sarcoma accounts for about 1% of all soft tissue sarcomas every year in the U.S.

Experts believe there are fewer than 1,000 people in the U.S. who currently have ES.

Who does epithelioid sarcoma affect?

Distal type epithelioid sarcoma (the most common type) typically affects teenagers and young adults. Proximal type epithelioid sarcoma (the rarer type) mainly affects adults.