Ewing sarcoma is a type of cancerous tumor that grows from cells in bones or in the soft tissue surrounding bones. It most frequently arises in the legs, pelvis, ribs, arms, spine or skull.

Ewing sarcoma typically occurs in adolescents or young adults between the ages of 10 and 20, and affects slightly more boys than girls. White and Hispanic populations are often most affected. While it is the second most common bone cancer among children, Ewing sarcoma diagnoses are actually pretty rare—only about 200 cases are diagnosed each year in the United States, making up 1 percent of all childhood cancers.

Common signs and symptoms of Ewing sarcoma include:

  • Pain and/or swelling near the growing tumor
  • Unexplained fever, fatigue or weight loss
  • Unexplained bone breaks, or bones breaking easily
  • The presence of a lump beneath the skin in areas like arms, legs, chest or pelvis

Because these symptoms are non-specific and could be the result of another illness or condition, Ewing sarcoma tumors may not be suspected or found right away. It is important to monitor for these symptoms, recognize when they are persisting, and to follow up with your doctor for further exams.

Diagnostic tests for Ewing sarcoma include MRIs, CT scans, X-rays, and PET scans. Blood tests such as a complete blood count (CBC) test and a bone marrow aspiration (in which a sample of bone marrow is removed for testing), with a subsequent biopsy, can also be used.

Treatment for Ewing sarcoma can include chemotherapy and radiation; surgery, including limb salvage surgery and rotationplast; and targeted therapy, such as monoclonal antibody therapy.

This article was originally published on July 9, 2018, by Dana-Farber Cancer Institute. It is republished with permission.