Pediatric Ewing Sarcoma

How is pediatric Ewing sarcoma diagnosed?

If your child has symptoms of ES, the doctors at Riley at IU Health can perform the following exams and tests to make a diagnosis:

• Physical examination. Diagnosing pediatric ES typically begins with a physical examination by your child’s doctor. Any abnormal findings may be further evaluated if there is concern for a tumor.
• X-ray. X-ray images of a concerning area are often the next step in diagnosing a tumor. In some cases, X-rays will demonstrate findings consistent with a growth in or near the bone, or they will demonstrate abnormal breaks in a child’s bones that warrant further evaluation.
• Magnetic resonance imaging (MRI). MRI is an imaging procedure that uses a magnetic field instead of radiation to show tumors in bone, muscle and soft tissue. MRI allows doctors to evaluate the area of concern in greater detail.
• Computed tomography (CT). CT creates multiple detailed or three-dimensional images of bone, tissue and blood vessels. Doctors may also use this fast, painless imaging procedure to evaluate the area of concern.
• Blood tests. A blood test may be ordered to look at the red blood cells, white blood cells, platelets and blood chemistries that can be affected in children with cancers.
• Biopsy. Ultimately, a sample of the tumor itself (biopsy) is needed to confirm the diagnosis of pediatric ES. Depending on the location and size of the tumor, a biopsy can either be done with a needle, by surgical removal of a small piece of the tumor or, in some cases, by surgical removal of the entire tumor. The pieces of tumor are then specially prepared and placed on microscope slides for evaluation by the pathologist who provides a final tissue diagnosis.

Once a tumor has been diagnosed as ES, the cancer team will obtain additional tests to determine if the tumor has spread to other areas of the body. This is called staging and may include:

• Chest X-ray
• Bone scans
• Additional MRI or CT imaging
• Bone marrow biopsy
• Bone marrow aspiration

How is pediatric Ewing sarcoma treated?

The treatment of ES is likely to include a combination of:

• Chemotherapy. Chemotherapy is when medicines are provided by mouth or intravenously to destroy cancer cells.
• Radiation therapy. High-energy radiation is directed on the tumor to shrink or destroy it or to kill cancer cells.
• Surgery. The tumor and/or affected bone or tissue may be removed depending on the location and advancement of ES.

The choice of therapies, timing and duration will be chosen by a team of oncologists (cancer doctors) based on the original site of the tumor and whether it has spread at the time of diagnosis.

A number of factors can influence a child’s long-term outlook, including:

• Extent of the condition (whether the tumor has spread to nearby or distant sites in the body)
• Location of the initial tumor
• Size of the tumor at diagnosis
• Tumor genetics
• Patient’s age and gender
• Tumor response to therapy
• Whether the tumor was completely removed

In general, the overall five-year survival for localized ES (that has not spread at the time of diagnosis) is favorable. About 70 percent of children are alive at five years after their diagnosis. Because this percentage is based on survival after five years, it does not reflect the improved prognosis that can come from recent improvements in treatment.

Key Points to Remember

• Pediatric Ewing sarcoma (ES) is a form of cancer/tumor that primarily affects children and young adults.
• Pain in the area of the tumor that does not improve at night is the most common symptom.
• Many symptoms of ES are similar to common complaints in an active child or adolescent, making a diagnosis difficult based on symptoms alone.
• Ultimately, a sample of the tumor itself (biopsy) is needed to confirm the diagnosis of ES.
• Therapy will likely include a combination of chemotherapy, radiation therapy and surgery.