Ewing Sarcoma

Pediatric Cancers - Ewing Sarcoma

What is Ewing sarcoma?

Ewing sarcoma is a cancer that occurs primarily in the bone or soft tissue. Ewing sarcoma can occur in any bone, but it most often it is found in the long bones such as the femur (thigh), tibia (shin), or humerus (upper arm). It can involve the muscle and the soft tissues around the tumor site as well. Ewing sarcoma cells can also spread (metastasize) to other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland, and other soft tissues.

Ewing sarcoma accounts for about 2 percent of childhood cancers. About 225 children and adolescents are diagnosed with Ewing sarcoma in the U.S. each year. It is the second most common malignant bone tumor in children and adolescents. Ewing sarcoma most often occurs in children between the ages of ten and 20. More males are affected than females.

What causes Ewing sarcoma?

The exact cause of Ewing sarcoma is not fully understood. There seems to be no known risk factors or prevention measures available. However, researchers have discovered chromosomal changes in a cell's DNA that can lead to Ewing sarcoma formation. These changes are not inherited. They develop in children after they are born for no apparent reason.

In nearly 85 percent of these cases, the change involved the fusing of genetic material between chromosomes #11 and #22. When a certain piece of chromosome #11 is placed next to the EWS gene on chromosome #22, the EWS gene gets "switched on." This activation leads to an overgrowth of the cells and eventually the development of cancer. The exact mechanism remains unclear, but this important discovery has led to improvements in diagnosing Ewing sarcoma.

Some physicians classify Ewing sarcoma as a primitive neuroectodermal tumor (PNET). This means the tumor may have started in fetal, or embryonic, tissue that has developed into nerve tissue.

What are the symptoms of Ewing sarcoma?

The following are the most common symptoms of Ewing sarcoma. However, each child may experience symptoms differently. Symptoms may include, but are not limited, to the following:

The symptoms of Ewing sarcoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is Ewing sarcoma diagnosed?

In addition to a complete medical history and physical examination of your child, diagnostic procedures for Ewing sarcoma may include:

Ewing sarcoma is difficult to distinguish from other similar tumors. Diagnosis is often made by excluding all other common solid tumors, and by the use of genetic studies.

Treatment for Ewing sarcoma:

Specific treatment for Ewing sarcoma will be determined by your child's physician based on:

Treatment may include one, or more, of the following:

Long-term outlook for a child with Ewing sarcoma:

Prognosis for Ewing sarcoma greatly depends on:

A person who was treated for Ewing sarcoma as a child or adolescent may develop effects months or years after treatment ends. These effects are called late effects. The kind of late effects one develops depends on the location of the tumor and the way it was treated.

Some types of treatment may later affect fertility. If this side effect is permanent, it will cause infertility, or the inability to have children. Both men and women can be affected.

As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structured around the child's needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with Ewing sarcoma. Late effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of Ewing sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

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