Ewing’s sarcoma is a cancer that occurs primarily in the bone or soft tissue. Ewing sarcoma can occur in any bone, but is most often found in the extremities and can involve muscle and the soft tissues around the tumor site. Ewing’s 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’s sarcoma is the second most common malignant bone tumor in children and adolescents, although it occurs in the soft tissues as well. Ewing’s sarcoma most often occurs in children between the ages of ten and 20. The number of males affected is slightly higher than the number of females.

 

The majority of Ewing’s sarcomas result from a chromosome rearrangement between chromosomes #11 and #22. This rearrangement changes the position and function of genes, causing a fusion of genes referred to as a "fusion transcript." Over 90 percent of individuals have an abnormal fusion transcript, involving two genes known as EWS and FLI1. This important discovery has led to improvements in diagnosing Ewing’s sarcoma.

 

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’s sarcoma. Late effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of Ewing’s sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

 

Source: Lucile Packard Children’s Hospital