• Ewing Sarcoma Overview

    Ewing sarcoma is a type of cancer that grows in bones or soft tissue around bones, primarily in the thigh, shin and upper arm. These tumors can also affect muscle and tissue around the area of the tumor and can spread to other bones, the lung and sometimes to the kidney or adrenal gland.

    Ewing sarcoma affects children (boys more than girls) and young adults from ages 5 to 20 and is rare in children of African descent. 

    Ewing Sarcoma Treatment at Dana-Farber/Boston Children's

    Children and young adults with Ewing sarcoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Bone & Soft Tissue Program. Continue reading to learn more about Ewing sarcoma or visit the Bone & Soft Tissue Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    The symptoms of Ewing sarcoma are often non-specific, including:

    • Localized pain and swelling
    • Sporadic bone pain of varying intensity
    • Limited range of motion in the affected area
    • Fever of unknown cause

    Doctors diagnose Ewing sarcoma with:

    • Magnetic resonance imaging (MRI)
    • Computerized tomography (CT or CAT) scan
    • Positron emission tomography (PET) scan
    • Biopsy
    • Bone scan to determine the cause of pain and inflammation

    Less often, genetic testing and bone marrow aspiration (analysis of a small amount of fluid from bone marrow) can sometimes help confirm a diagnosis. After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Treatments for Ewing sarcoma have improved dramatically in recent years. Typically, the larger the bone, the harder the disease is to treat. Even if tumors originate in small bones, however, the disease may spread, requiring treatment across the entire body. 

    Local control of Ewing sarcoma is achieved with surgery and/or radiation therapy. Because of the high likelihood that the cancer will spread, local control is only effective in about 20 percent of cases. Chemotherapy is the first-line treatment for Ewing sarcoma that has spread elsewhere in the body.

    Surgery may be performed after chemotherapy to remove any remaining parts of the tumor. Whenever possible, surgeons try to avoid amputation by removing only the cancerous section of bone and replacing it with an artificial device called an endoprostheses. When surgical removal is difficult, targeted radiation therapy can be an effective treatment option.

    An autologous stem cell transplant is a potentially promising treatment.

    Progressive or Recurrent Disease

    Nearly 80 percent of Ewing sarcoma recurs within two years of the initial diagnosis. Late relapses—more than five years from initial diagnosis—have been reported. Different combinations of chemotherapy drugs are used to treat recurrent disease. There is no standardized second-line treatment for relapsed or refractory Ewing sarcoma.

    Long-term Outlook

    With early diagnosis and proper treatment, many children and young adults with Ewing sarcoma have a good chance of recovery, with a five-year survival rate near 80 percent for localized tumors. The overall survival rate for recurrent Ewing sarcoma, however, is poor. Children whose disease recurs more than two years after diagnosis have a five-year survival rate of 30 percent, while those whose disease recurs less than two years from diagnosis have only a 7 percent five-year survival rate.
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    Fernando Morales, a Ewing sarcoma, patient was a good match for an innovative treatment that allowed him to spend more time at home during chemotherapy treatments.