Ewing Sarcoma in Children

Ewing sarcoma is a cancer that occurs primarily in the bone or soft tissue. It 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. It accounts for about 2–3% of childhood cancers.

About 150 children and adolescents are diagnosed with Ewing sarcoma each year in the U.S. It is the second most common malignant bone tumor in children and adolescents and accounts for about 30% of pediatric bone cancers.

Ewing sarcoma most often occurs in children between 10- and 20-years-old. Before adolescence, the number of males and females affected are equal. After adolescence, however, the number of males affected is slightly higher than the number of females. It has been suggested that the increased rate of growth among males during adolescence may account for this increased incidence.

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% 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.

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 the procedures listed below.

X-Rays

A diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. This test is used to measure and evaluate the curve.

Radionuclide Bone Scan

Pictures or x-rays taken of the bone after a dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.

Magnetic Resonance Imaging (MRI)

A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test is done to rule out any associated abnormalities of the spinal cord and nerves.

Computed Tomography Scan (CT or CAT Scan)

A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.

Biopsy of the Tumor

A procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope; to determine if cancer or other abnormal cells are present; to remove tissue from the affected bone.

Bone Marrow Aspiration and/or Biopsy

A procedure that involves a small amount of bone marrow fluid and tissue to be taken, usually from part of the hip bones, to further examine the number, size, and maturity of blood cells and/or abnormal cells; to detect cancer cells. 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.

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:

• Pain around the site of the tumor
• Swelling and/or redness around the site of the tumor
• Fever
• Weight loss, decreased appetite
• Fatigue
• Paralysis and/or incontinence (if the tumor is in the spinal region )
• Symptoms related to nerve compression from tumor (i.e., numbness, tingling, paralysis, etc.)

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

What Is the Treatment for Ewing Sarcoma?

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

• Your child's age, overall health, and medical history
• Extent of the disease
• Your child's tolerance of specific medications procedures, or therapies
• Expectations for the course of the disease
• Your opinion or preference

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

• Surgery
• Chemotherapy
• Radiation therapy
• Amputation (not usually necessary)
• Resections for metastases (i.e., pulmonary resections of cancer cells in the lung)
• Rehabilitation, including physical and occupational therapy, and psychosocial adaptation
• Prosthesis fitting and training
• Supportive care (for the side effects of treatment )
• Antibiotics (to prevent and treat infections )
• Continual follow-up care (to determine response to treatment, detect recurrent disease and manage late effects of treatment)

What Are the Long-term Effects of Ewing Sarcoma?

Prognosis for Ewing sarcoma greatly depends on:

• The extent of the disease
• The size and location of the tumor
• Presence or absence of metastasis
• The tumor's response to therapy
• The age and overall health of your child
• Your child's tolerance for specific medications, procedures, or therapies
• New developments in treatment

As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique, and treatment and prognosis are 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.