Ewing's Sarcoma

Our multidisciplinary team of experts who provide medical care for Ewing sarcoma patients includes pediatric oncologists, medical oncologists, orthopedic oncologists (orthopedic surgeons who specialize in bone and soft tissue tumors), pediatric surgeons, radiation oncologists, plastic surgeons, vascular surgeons and rehabilitation experts.

Diagnosis: The first sign of Ewing sarcoma may be pain in a bone or joint. The individual may think the pain and swelling comes from a sports injury or sprain. However, a doctor should evaluate the symptoms when pain does not go away in a reasonable amount of time. Evaluation of the patient begins with imaging studies, such as X-ray, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI), to locate the suspected tumor.
Other imaging techniques that may be used include a chest CT, a bone scan and positron emission tomography (PET) scan. These images are helpful in evaluating the lungs and bones, areas to which the cancer commonly spreads (metastasizes). To diagnose Ewing's sarcoma, the doctor removes small samples of the suspected tumor for examination (biopsy). A pathologist looks at the cells under a microscope to determine whether the tissue contains malignant cells. In some cases, tumor cells are tested for chromosome abnormalities. A bone marrow biopsy may also be needed to help doctors determine the extent of the disease.

The treatment of patients with Ewing sarcoma includes a combination of cancer-fighting drugs (chemotherapy) and surgery or radiation. The treatment plan has two main goals: (1) to cure the cancer, and (2) to save as much function of the affected area as possible. Patients with Ewing sarcoma receive a combination of cancer-fighting drugs (chemotherapy) and surgery or radiation. Oncologists prescribe chemotherapy to control the spread of the disease to other bones or locations in the body and to shrink the tumor. Surgery or radiation typically is scheduled two to four months after chemotherapy begins. The decision to use radiation, surgery or both can be difficult, and the choice varies among patients. Following surgery or radiation, physicians typically recommend additional chemotherapy to eliminate any remaining cancer cells.  In about 80 percent of Ewing sarcoma cases involving an arm or leg, the limb can be saved. We have significant experience in limb-saving techniques, including bone grafts, rotationplasty and prosthetic implants.

Limb Salvage Techniques: The possibility of saving the limb depends on the tumor's size and location; whether it has grown into or around a joint, nerves, or blood vessels; and its response to chemotherapy.Options include rotationplasty, bone grafts (autografts and allografts) and prosthetic implants.

Autograft: In this procedure a surgeon harvests the patient's own bone tissue to implant at the site where Ewing's sarcoma tumor is removed. One common form of this procedure is called free vascularized fibular grafting. In this procedure surgeons harvest the smaller of the two lower leg bones and its blood vessels to implant at the cancer site. We have used the fibula to replace the upper leg bone, the upper arm bone, the lower leg bone and the jaw. Benefits of this procedure over allograft (bone graft from a donor) include a greater chance for the bone to heal and a reduced chance for infection.

Allograft: Orthopedic oncologists can use bone, tendons and ligaments from a tissue bank to rebuild areas where the Ewing sarcoma tumor is removed. This procedure is called an allograft. We have significant experience using the procedure to replace joints (osteoarticular allograft) or other portions of the musculoskeletal system.

Rotationplasty: This procedure is typically used for young or athletic patients who have Ewing sarcoma near the knee joint. Rotationplasty can allow a patient to run or walk with near-normal function. During the procedure, an orthopedic surgeon removes the tumor by cutting above and below the knee. The lower portion of the leg is rotated and reattached so that the ankle "becomes" the knee. Specialists then fit the patient with a prosthetic ankle and foot.

Prosthetic Implants: Surgeons can replace sections of bone with specialized prosthetic implants. Expandable and solid implants of many different sizes are available. Surgeons consider whether the patient will experience further bone growth before recommending prosthetic implants.