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Ewing sarcoma is the second most common tumor of the bone. It occurs most frequently in the long bones of the legs or arms, the pelvis, chest wall, spine and the skull. Ewing sarcoma can also begin in the soft tissues. This disease most often occurs in adolescents, with nearly half of cases arising between the ages of 10 and 20.

This group of tumors includes Ewing sarcoma, atypical Ewing sarcoma and peripheral primitive neuroectodermal (PNET) of bone. These tumors are sometimes referred to as the Ewing family of tumors.
Ewing sarcoma and related tumors represent 2-3% of all children’s cancers.
About 250 children and adolescents are diagnosed with Ewing sarcoma or related tumors in the United States each year.

The symptoms of Ewing Sarcoma depend on the bone in which the cancer develops, but usually include:

Pain at the site of the mass, with soft tissue swelling around the mass
In cases of metastatic disease (where the cancer has spread), patients may have general symptoms such as loss of appetite, fever, malaise, fatigue, and weight loss
Other symptoms related to the specific location of the tumor

Quite often a child’s regular doctor may have already performed some type of x-ray; such exams are often repeated by the specialist. If a bone tumor is suspected, it is important that the child is diagnosed and treated by a team experienced in dealing with children with cancer.

The most important evaluation of the tumor is the biopsy. In this test, a sample of tumor tissue will be removed from the site and examined under a microscope by a pathologist.

Determining the Extent of Disease

In patients with Ewing Sarcoma, 10% to 30% have metastatic disease at the time of diagnosis, meaning the cancer has spread to other areas. Staging the disease—determining how far it has spread—is very important because it helps the healthcare team make the best, most informed decisions about treatment.

Some of the other examinations that may be performed include:

CT scan or MRI of the primary tumor
Chest x-ray
CT scan of the chest
Bone scan
Bone marrow aspirate and bone marrow biopsy

The extent of the disease at diagnosis is the most important factor in determining the prognosis (chance of recovery). Other factors include:

Primary site
The presence of metastasis
Size of the tumor
Age of the child

Causes of Ewing Sarcoma

Researchers currently DO NOT know what causes Ewing’s sarcomas. However, they do know that it is not associated with any other cancers and there is no additional risk among families in which a single person has Ewing Sarcoma.

Only a few risk factors for Ewing sarcomas are known for sure. Ewing sarcomas occur mostly in Caucasian and Hispanic children. Caucasian and Hispanic or Latino children are about 9 times more likely to develop Ewing sarcoma compared to African American children. Ewing sarcomas are rare in Chinese and Japanese children. More boys than girls develop Ewing sarcoma.

Last updated July, 2011

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