WHAT IS EWING SARCOMA? Ewing sarcoma is a type of cancerous tumor arising from the bone or soft tissues. Ewing sarcoma belongs to a group of tumors called the Ewing sarcoma family of tumors (EFT). EFT consists of • Ewing sarcoma of the bone • extraosseous Ewing sarcoma (EES) • Askin tumor • primitive neuroectodermal tumor (PNET) of the bone • PNET of the soft tissue. Under the microscope, tumors in this family appear similar and are made up of small round blue cells. These tumors are generally evaluated and treated the same way. To simplify the discussion, the term Ewing sarcoma will be used throughout this handbook to refer to any one of these tumors. In 1921, Dr. James Ewing became the first to describe Ewing sarcoma. After osteosarcoma, Ewing sarcoma is the most common type of childhood bone cancer. It is usually found in the pelvic bones, the upper arm, the spine, the ribs, or the femur (one of the long bones of the leg), but it can occur in any bone in the body. Ewing sarcoma found in the soft tissues around the bone is called extraosseous or extraskeletal Ewing sarcoma (EES). EES is most commonly found in the thigh, pelvis, spine area, chest wall, or foot. Ewing sarcoma found in the chest wall may be referred to as an Askin tumor . PNET is the least common member of EFT. It is also a round-cell tumor, but it is made up of young nerve cells. PNET can occur in the bone or soft tissues.
WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF EWING SARCOMA?
The most common symptom of Ewing sarcoma is pain at the site of the tumor. The pain may wake your child up from sleep. Swelling or a soft mass may appear around the affected bone or tissue. If the tumor is located in the pelvis, signs of bowel or bladder disturbance may appear. If the tumor is located in the spine, the patient often experiences back pain, weakness in the arms or legs, or numbness. If the tumor is located in the chest wall, symptoms may include cough, shortness of breath, or chest pain. A pathological fracture (a break that occurs without trauma) is often present at diagnosis because the tumor has weakened the bone. It is important to understand that trauma did not cause the cancer. Children frequently have painful lumps as a result of normal play, so Ewing sarcoma usually is diagnosed only after a lump has persisted for several months. Other less common symptoms of Ewing sarcoma are weight loss and fever.
WHAT CAUSES EWING SARCOMA? We do not know what causes Ewing sarcoma. We know that Ewing sarcoma is not contagious—that is, it cannot be caught from another person. For many adult cancers, lifestyle-related risk factors play a large role—but no behaviors or lifestyle habits, including those practiced during the mother’s pregnancy, have been associated with the development of Ewing sarcoma. Studies of children with Ewing sarcoma have not found links to radiation, chemicals, or other environmental factors, nor is Ewing sarcoma caused by too much or too little of a particular food group in a child’s diet.
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