What Is Ewing Sarcoma?

Steven Dubois, sarcoma, pediatric
Steven Dubois, MD, MS, is a leader in Dana-Farber/Boston Children’s sarcoma program.

Ewing sarcoma is a cancer that grows in the bones and soft tissues, usually in the thigh or pelvis. It is most common in children and young adults between the ages of 5 and 20, and more common in boys than girls. Although most patients are teenagers, Ewing sarcoma can sometimes occur in older adults in their 20s or 30s. These tumors account for 2 percent of all childhood cancers.

The symptoms of Ewing sarcoma are similar to symptoms of other, more common illnesses, but may include:

  • fever
  • sporadic bone pain, or pain around the tumor site
  • limited range of motion in the affected area
  • swelling or redness around the tumor site
  • decreased appetite and weight loss
  • fatigue

 

Doctors may diagnose Ewing sarcoma with MRI, PET, or bone scans, or with a bone marrow or tumor biopsy. These tests can also help determine the size of the tumor and whether it has spread elsewhere in the body.

Treatment for Ewing sarcoma usually begins with chemotherapy to shrink the cancer cells and prevent them from spreading. Surgeons may then be able to remove the remaining tumor through either limb-preserving surgery, amputation, or rotationplasty, depending on the size and location of the tumor. Radiation therapy may also be necessary following surgery, although some patients may have radiation therapy to shrink the tumor before, or instead of, surgery. Clinical trials may also be an option for patients with Ewing sarcoma, especially for those whose disease has metastasized, or spread, to other parts of the body, or for those whose cancer has recurred. Physical therapy or integrative therapies may help with recovery after surgery and other treatments.

Ewing sarcoma may recur later in life, so it is important to have long-term follow-up care, including imaging scans following treatment, through a survivorship clinic such as the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center.