Ewing sarcoma is a type of cancer that may be a bone sarcoma or a soft-tissue sarcoma. Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture. The most common areas where it begins are the legs, pelvis, and chest wall. In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis. Complications may include a pleural effusion or paraplegia.
The cause of Ewing sarcoma is unknown. Most cases appear to occur randomly. It is sometimes grouped together with primitive neuroectodermal tumors, in a category known as the Ewing family of tumors. The underlying mechanism often involves a genetic change known as a reciprocal translocation. Diagnosis is based on biopsy of the tumor.
Treatment often includes chemotherapy, radiation therapy, surgery, and stem cell transplant. Targeted therapy and immunotherapy are being studied. Five year survival is about 70%. A number of factors, however, affect this estimate.
James Ewing in 1920 established that the tumor is a distinct type of cancer. It affects about one in a million people per year in the United States. Ewing sarcoma occurs most often in teenagers and young adults and represents 2% of childhood cancers. Caucasians are affected more often than African Americans or Asians. Males are affected more often than females.