Ewing's Sarcoma is a very rare form of bone cancer that strikes children and adolescents. Actually, it is a group of four different types of cancer, known collectively as the Ewing's Family of Tumors (EFT). About 250 cases are diagnosed every year in the U.S. The good news is that two-thirds of patients are long-term survivors of the disease. Although it can occur at a younger age, most Ewing's sarcoma cases are found in children aged 10 to 20. Caucasian children, particularly boys, are much more likely to have EFT than any other racial or ethnic group.
Ewing's sarcomas are caused by chromosome changes that occur after birth. In a process known as translocation, chromosomes 11 and 22 "swap" small pieces of each other, resulting in an abnormal gene that can be detected with DNA testing. However, it's not known why this translocation takes place. There are no identifiable risk factors that increase a child's susceptibility to Ewing's sarcomas, and it's not passed along from parent to child like some inherited childhood cancers. In fact, it's extremely rare for it to happen more than once in the same family.
Ewing's bone sarcoma accounts for about 85% of all cases. It's usually found in the long bones in the arm or leg, although it sometimes occurs in the pelvis or ribcage.
Extraosseous sarcomas are tumors that occur outside the bone. Only 8% of EFT cases are of this type.
Primitive Neuroectodermal Tumor (PNET), also known as peripheral neuroepithelioma, can occur in bone and/or soft tissue.
Askin's tumor is a PNET that occurs in the marrow cavities of the chest wall. It often spreads to the surrounding soft tissue.
An X-ray is generally the first diagnostic test ordered when a child has bone pain or other symptoms. While X-rays can detect the tumor, the pediatric oncologist may order another imaging test such as an MRI or CT scan to get more detailed information about the tumor location and whether or not it has spread. A bone biopsy may also be done to ensure a correct diagnosis, since other cancers may affect bone. Although Ewing's sarcomas are rare, MD Anderson pediatricians see many of these patients and have the expertise to diagnose and treat these tumors.
Children with Ewing's Sarcomas undergo intensive cancer treatment. Once diagnosed, the child is immediately put on a multi-drug chemotherapy regimen. After about nine weeks, the patient will either undergo surgery to remove what's left of the tumor, or radiation therapy if surgery is not possible. After that, the chemotherapy is continued for a total of up to nine months.
In the past, surgery on tumors in the arm or leg bones almost always required amputation. Limb-sparing surgery techniques, such as bone grafts and artificial bones, can help certain patients avoid losing an arm or leg. The outlook for many Ewing's Sarcoma patients is optimistic, except in cases where large tumors cannot be completely removed or have metastasized (spread) to other parts of the body.