Key Points about Epithelioid Sarcoma

  • This is a type of soft tissue sarcoma; there are two main types of epithelioid sarcoma.
  • Doctors use imaging tests, biopsy and physical exams to diagnose epithelioid sarcoma.
  • Treatment for epithelioid sarcoma may include surgery, chemotherapy, targeted drug therapy and/or radiation therapy.


Epithelioid sarcoma is a rare type of soft tissue sarcoma that usually begins in the soft tissue of the skin of a finger, hand, forearm, foot or lower leg. There are two types of epithelioid sarcoma:

  • Distal-type epithelioid sarcoma – this classic type most often occurs in teenagers and young adults.
  • Large-cell epithelioid sarcoma – this much rarer type usually affects adults and grows more quickly.

Epithelioid sarcoma causes

Epithelioid sarcoma is caused when mutations (changes) occur in the soft tissue cells, and then those cells grow and multiply quickly.

Epithelioid sarcoma risk factors

The following factors may increase your risk for developing epithelioid sarcoma:

  • Having specific inherited genetic syndromes, including:
    • Li-Fraumeni syndrome
    • Familial adenomatous polyposis
    • Neurofibromatosis
    • Tuberous sclerosis
    • Werner syndrome
  • Having been exposed to certain harmful chemicals, including herbicides, dioxin or arsenic
  • Having undergone radiation therapy

Epithelioid sarcoma symptoms

In its early stages, epithelioid sarcoma typically doesn’t cause any symptoms. As it grows, the sarcoma may cause:

  • A lump or area of swelling
  • Pain (if the tumor is pressing on nerves or muscles)

Epithelioid sarcoma diagnosis

Your oncologist may use one or more of the following tests to diagnose this condition:

  • Physical exam – your doctor will perform a complete physical exam, including asking questions about your health history, symptoms and related risk factors.
  • Biopsy – in this test, your doctor removes a biopsy (small sample) from the suspicious area. This sample is sent to the laboratory, where a specialist closely checks the biopsy for epithelioid sarcoma.
  • Computerized tomography (CT) scan – this type of imaging test provides a 3D image of the inside of the body that your doctor can use to determine the size and location of the epithelioid sarcoma.
  • Magnetic resonance imaging (MRI) – this type of imaging test uses high-powered magnets to create detailed images of the inside of your body. Your doctor can closely examine these images to determine the size and location of the epithelioid sarcoma.

Epithelioid sarcoma treatments

Depending on your personal health history, the extent of the cancer and other factors, your oncologist may recommend one or more of the following treatment options:

  • Surgery – you may need to undergo surgery to remove the cancerous area. Your surgeon will work to preserve as much surrounding healthy tissue as possible.
  • Chemotherapy – you may need to also undergo chemotherapy to destroy any cancerous cells that couldn’t be removed surgically. During this treatment, medication is used to destroy cancerous cells. Chemotherapy can be taken via an oral (by mouth) pill or intravenously (through a vein).
  • Radiation therapy – this treatment uses high-powered energy beams to destroy cancerous cells. You may need to undergo radiation therapy to destroy any cancerous cells that couldn’t be removed surgically.

When should I seek care?

If you experience any of these symptoms, start by voicing your concerns and symptoms to your primary care provider. Your doctor may suggest seeing an oncologist for more specialized treatment.

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