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 is most often found in the extremities and can involve muscle and the soft tissues around the tumor site. 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 to 3 percent of childhood cancers. About 250 children and adolescents are diagnosed with Ewing sarcoma each year in the US. 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. The number of males affected is slightly higher than the number of females.
What causes Ewing sarcoma?
The majority of Ewing 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 sarcoma.
Similar to osteogenic sarcoma, trauma or injury is sometimes involved with the site at the time of diagnosis. However, this trauma is thought to bring the condition to attention rather than to have any causal relationship.
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?
Children’s Hospital of Orange County is a leader in diagnosing and treating childhood bone cancer. The CHOC Cancer Institute takes an advanced approach to this condition, offering patients access to the latest treatment alternatives in a compassionate, patient-centered environment.
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.
Stages of Ewing’s Sarcoma:
Once Ewing’s sarcoma is found, more tests will be done to determine if the cancer cells have spread to others parts of the body. This is called staging. Currently, there is no formal staging system for the Ewing’s family of tumors. Instead, most patients are grouped depending on whether cancer is found in only one part of the body (localized disease) or whether cancer has spread from one part of the body to another (metastatic disease). Treatment is tailored to where the cancer is located and how far the disease has spread.
The following groups are used for the Ewing’s family of tumors:
Treatment for Ewing sarcoma:
Specific treatment for Ewing sarcoma will be determined by your child's physician based on:
The most common treatments for Ewing’s sarcoma are surgery, radiation therapy and chemotherapy.
A supplement to the treatment options listed above is myeloablative therapy with stem cell support. Myeloablative therapy is a very intense regimen of chemotherapy designed to destroy all cells that divide rapidly. These cells include some blood cells and hair cells, as well as malignant cancer cells. Stem cells are self-renewing cells that create all of the other various types of blood cells. Stem cell support involves enriching the stem cells to increase the number of these important cells circulating in the blood after the chemotherapy has been given to kill the remaining tumor cells.
Additional 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:
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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It is important to remember the health information found on this website is for reference only not intended to replace the advice and guidance of your healthcare provider. Always seek the advice of your physician with any questions you may have regarding a medical condition. If you think you may have a medical emergency, call your physician or 911 immediately.
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