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Epithelioid sarcoma (ES) is a rare type of soft tissue sarcoma. This is a kind of cancer that grows in soft tissues of the body like muscles, fat or blood vessels.
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Types of Epithelioid Sarcoma
There are two main types of epithelioid sarcoma:
- Distal epithelioid sarcoma: Usually appears on the hands, arms or feet.
- Proximal type epithelioid sarcoma: It is less common than distal epithelioid sarcoma. It is found closer to the center of the body, like the chest or back.
Symptoms and Signs
The most common sign of epithelioid sarcoma is a hard, slow-growing firm lump under the skin. Most of the time, these lumps do not hurt. However, they can cause pain if they grow near a joint.
Another sign is if the skin covering the lump looks shiny or has a tan or grayish color. In some cases, the lump can turn into an open sore that does not heal normally. These growths are most likely to show up on the fingers, hands and forearms. But they can also be found on the legs and knees.
Causes and Risk Factors
There is no known cause for epithelioid sarcoma. While tumors sometimes appear in places where a past injury has occurred, there is no proof that an injury actually causes the disease. Scientists have found that in most patients, the cancer starts when there is a gene error and the cells grow and multiply when they should not. This eventually creates a tumor.
Epithelioid sarcoma is more common in men than in women and is most likely to affect people between age 10 and 45.
Epithelioid Sarcoma Care Team
At Fred Hutch, we offer comprehensive treatment from a team of experts who specialize in soft tissue sarcomas and bone cancers.
Learn more about sarcoma specialists at Fred Hutch.
Diagnosing Epithelioid Sarcoma
Because this cancer is rare and grows slowly, it can sometimes take a long time to get a correct diagnosis. In order to diagnose epithelioid sarcoma, a physician will start by doing a physical exam to feel for any unusual lumps. They will then order imaging scans. This might include an MRI, CT or PET-CT, to see further examine the tumor and see if it has spread. The most important step to diagnose this disease is a biopsy, where a physician removes a tiny sample of the lump to send to a lab so it can be examined. Specialists will test this sample to make sure the diagnosis is accurate and use this information to help stage the disease.
Learn more about diagnosing and staging sarcoma.
Treating Epithelioid Sarcoma
The main treatment for epithelioid sarcoma is surgery. A physician may also recommend radiation therapy, which uses high-energy rays to destroy any cancer cells that might remain. For cases where the cancer has spread to other parts of the body, other treatments called targeted therapies might be used. Because this type of cancer has a high chance of returning even after treatment, patients need to have regular check-ups and scans after treatment.
Learn more about treating sarcoma.