Ewing = James Ewing (1866-1943), who first described this type of cancer
Sarcoma = cancer of a bone or soft tissue (muscle, tendon, cartilage, nerve, blood vessel, or others)
Ewing sarcoma is a cancer of the bones or soft tissues. It is most common in the pelvis and the large long bones of the arms and legs, but it can also occur in the other bones of the limbs, the neck, clavicle, ribs, and spine. At the Spine Hospital at the Neurological Institute of New York, we specialize in treating Ewing sarcoma of the spine.
Ewing sarcoma is sometimes also called Askin tumor or peripheral primitive neuroectodermal tumor.
Ewing sarcoma of the spine often causes pain at the site of the tumor, followed by a palpable swelling. If the tumor grows large enough to compress the spinal cord, neurological symptoms such as weakness, clumsiness, or tingling may develop in the arms and legs.
Metastatic disease (a spread of the cancer) may cause fever and weight loss.
Causes and Risk Factors
The overall rate of Ewing sarcoma in the United States is 1 case per 1 million people each year. Most patients are under 20 years old. Ewing sarcoma is slightly more common in males than females, and much more common in individuals with Caucasian ancestry than individuals with other ancestry.
A specific chromosomal translocation (a type of genetic mutation) is found in the cells of approximately 95% of Ewing sarcomas. However, the cause of this translocation is not known.
Tests and Diagnosis
Ewing sarcoma is a rare disease. It can occur anywhere in the body and its symptoms are nonspecific, or similar to the symptoms of many other problems. This disease is often misdiagnosed at first.
X-ray, MR (magnetic resonance) scan, CT (computed tomography) scan, PET (positron emission tomography) scan, bone scan, and biopsy all help to diagnose Ewing sarcoma.
- X-ray (also known as plain films) –test that uses invisible electromagnetic energy beams (X-rays) to produce images of bones. Soft tissue structures such as the spinal cord, spinal nerves, the disc and ligaments are usually not seen on X-rays, nor on most tumors, vascular malformations, or cysts. X-rays provide an overall assessment of the bone anatomy as well as the curvature and alignment of the vertebral column. Spinal dislocation or slippage (also known as spondylolisthesis), kyphosis, scoliosis, as well as local and overall spine balance can be assessed with X-rays. Specific bony abnormalities such as bone spurs, disc space narrowing, vertebral body fracture, collapse or erosion can also be identified on plain film X-rays. Dynamic, or flexion/extension X-rays (X-rays that show the spine in motion) may be obtained to see if there is any abnormal or excessive movement or instability in the spine at the affected levels.
- CT scan: procedure that uses a combination of X-rays and computer technology to produce detailed images of the body. CT scans are more detailed than general X-rays.
- PET scan: procedure that uses a very small amount of a radioactive material called a tracer to look for disease in organs or tissues.
- Bone scan: procedure in which a radioactive substance is injected into the body to measure activity in the bones. (The amount of radiation is small–less than the radiation in half of one CT scan.) This scan helps identifies damaged bones.
- Biopsy: procedure in which a small amount of tissue is removed for examination in a laboratory.
Ewing sarcoma is treated with a combination of chemotherapy, surgery, and sometimes radiation therapy.
The goal of surgical treatment is complete tumor removal. Depending on the location of the tumor, different portions of the vertebrae may need to be removed. To maintain spinal stability after diseased sections of bone have been removed, it is usually necessary to perform an instrumented spinal fusion.
Surgery for Ewing sarcoma of the spine should be performed by experienced cancer surgeons who specialize in spinal tumors.