Ewing sarcoma is a rare cancerous tumor of bone or soft tissue. It occurs mostly in young people.

Overall, it affects 1 out of every 1 million Americans. For adolescents ages 10 to 19 years, this jumps to about 10 cases per 1 million Americans.

The name comes from the American doctor James Ewing, who first described the tumor in 1921. It is not clear what causes Ewing sarcoma, so there are no known ways to prevent it. The condition is treatable, and full recovery is possible if it’s caught early.

Keep reading to learn more about Ewing sarcoma and its survival rate, symptoms, and treatments.

The 5-year survival rate for people who have localized tumors is about 80 percent. However, when the cancer has spread to other organs, the 5-year survival rate is about 38 percent.

Factors that can affect the survival rate include:

  • the size of the initial tumor
  • location of the tumor
  • the age of the patient

On average, 1 in 5 people with recurrent Ewing sarcoma survive long term. This outlook can vary based on the time between recurrences and the location of new tumors.

There are three types of Ewing sarcoma. These types depend on whether the cancer has spread from the bone or soft tissue where it started.

  • Localized Ewing sarcoma. The cancer has not spread to other areas of the body.
  • Metastatic Ewing sarcoma. The cancer has spread to the lungs or other places in the body.
  • Recurrent Ewing sarcoma. The cancer does not respond to treatment or returns after a successful course of treatment. It most often recurs in the lungs.

The most common symptom of Ewing sarcoma is pain or swelling in the area of the tumor.

Some people may develop a visible lump on the surface of their skin. The affected area may also feel warm to the touch.

Other symptoms include:

Tumors typically form in your:

  • arms
  • legs
  • pelvis
  • chest

There may be symptoms specific to the location of the tumor. For example, you may experience shortness of breath if the tumor is in your chest.

The below image shows an Ewing sarcoma on the left knee of a patient.

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If you or your child experience symptoms, reach out to a doctor. In about 25 percent of cases, the disease has already spread by the time of diagnosis. The sooner a diagnosis is made, the more effective treatment may be.

To diagnose Ewing sarcoma, a doctor will use a combination of the following diagnostic tests.

Imaging tests

Imaging tests may include one or more of the following:

  • X-rays. X-rays provide an image of your bones and can identify the presence of a tumor.
  • MRI scan. MRI scans take images of soft tissue, organs, muscles, and other structures. They show details of a tumor or other abnormalities.
  • CT scan. Doctors use CT scans to image cross-sections of bones and tissues.
  • EOS imaging. This procedure shows the interaction of joints and muscles while you’re standing.
  • Bone scan. A doctor may order a bone scan of your whole body to show if a tumor has metastasized, or spread.
  • PET scan. A PET scan shows whether any abnormal areas that were seen in other scans are tumors.


After imaging, your doctor can order a biopsy to look at a piece of the tumor under a microscope to identify it.

If the tumor is small, your surgeon may perform an excisional biopsy. In this procedure, surgeons will remove the whole tumor while the patient is under general anesthesia. If the tumor is larger, they may cut away a piece of it.

Your surgeon may also insert a needle into the bone to take out a sample of fluid and cells to see if the cancer has spread into your bone marrow.

After the removal of tumor tissue, there are several tests that help identify Ewing sarcoma. Blood tests may also provide helpful information for treatment.

The exact cause of Ewing sarcoma is not clear. It’s not inherited, but it can be related to noninherited changes in specific genes during a person’s lifetime. When chromosomes 11 and 12 exchange genetic material, it activates an overgrowth of cells. This may lead to the development of Ewing sarcoma.

Who is at risk for Ewing sarcoma?

Although Ewing sarcoma can develop at any age, more than 50 percent of people with the condition receive a diagnosis as adolescents. The median age of those affected is 15.

In the United States, Ewing sarcoma is more likely to develop in white people than Black and Asian people.

Treatment for Ewing sarcoma depends on:

  • where the tumor originated
  • the tumor’s size
  • whether the cancer has spread

Typically, treatment involves one or more approaches, including:

Treatment options for localized Ewing sarcoma

The common approach for cancer that has not spread is a combination of:

  • surgery to remove the tumor
  • radiation to the tumor area to kill any remaining cancer cells
  • chemotherapy to kill possible cancer cells that have spread, or micrometastasis

Further treatment may be necessary after surgery to replace or restore limb function, depending on where the tumor was.

Treatment options for metastasized and recurrent Ewing sarcoma

There is currently no standard treatment for recurrent Ewing sarcoma. Treatment options vary depending on where the cancer returned and what the previous treatment was.

Many clinical trials and research studies are ongoing to improve treatment for metastasized and recurrent Ewing sarcoma. These trials and studies include:

  • stem cell transplants
  • immunotherapy
  • targeted therapy with monoclonal antibodies
  • new drug combinations

As new treatments develop, the outlook for people affected by Ewing sarcoma continues to improve. A doctor is your best resource for information about your individual outlook and life expectancy.

There are many factors that may affect your individual outlook, including:

  • age at diagnosis
  • tumor size
  • tumor location
  • how well your tumor responds to chemotherapy
  • blood cholesterol levels
  • previous treatment for a different cancer
  • sex assigned at birth

Doctors will continue to monitor you during and after treatment. Your doctor will periodically retest to determine whether the cancer has spread. You may require checkups every 2 to 3 months in the years immediately after treatment.

Ewing sarcoma may return, and after treatment, you may have a slightly higher risk of developing a second kind of cancer later in life, according to Macmillan Cancer Support. These regular tests are the best way of catching any future developments early and improving your overall outlook.