Pediatric Ewing sarcoma (ES) is a form of cancer/tumor that primarily affects children and young adults. It is a rare cancer that typically involves the bones or the soft tissues surrounding the bones. It can occur in any bone, but it most frequently occurs in the hipbones and leg bones. Less frequently, ES can be found in the upper arms, shoulder blade, ribs, spine or skull bones. ES can start in the soft tissues that surround the bone. If ES has spread in the body (by a process called metastasis), it will most commonly spread to the lungs, distant bones or to the bone marrow.
ES is not an inherited cancer that is passed down from generation to generation. It is caused by genetic changes that have occurred in the cancer cells themselves. Most commonly, ES is the result of an abnormal joining of two genes that are normally separate.
The differences between pediatric and adult ES are many, and many of the differences are not well defined. It is important to know that pediatric and adult cancers should not be compared directly (diagnosis, treatment and outcome) as they are very different diseases with the same name.
Even though pediatric ES is the second most common pediatric bone cancer and makes up 2 to 4 percent of all childhood tumors, it remains rare overall.
Pediatric ES comprises a group of bone and soft tissue tumors known as the Ewing family of tumors that share a number of molecular and genetic characteristics but originate from different locations and may be treated differently. This group includes:
Symptoms of pediatric ES may include:
Less frequent symptoms include weight loss, infection and generalized bone pain.
Symptoms develop as the tumor grows. Some children will develop symptoms early, while others may not develop noticeable symptoms. Children who do not develop symptoms may be diagnosed when their doctor is performing an exam or test for another condition.
Many of the symptoms of pediatric ES are similar to common complaints in an active child or adolescent, making the diagnosis of ES difficult based on symptoms alone. Most children will experience symptoms on average for three to nine months prior to being definitively diagnosed.
Both boys and girls can be diagnosed with ES, but boys are diagnosed slightly more often than girls. Caucasian children develop ES more frequently than Asian-American or African-American children. Although ES can be diagnosed at any age, the majority of cases are diagnosed between 10 and 20 years of age for both boys and girls. ES is uncommon in children who are less than 5 years old, but it has been known to occur in this age group.
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:
Once a tumor has been diagnosed as ES, the oncology 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 a chest X-ray, bone scans, additional MRI or CT imaging, a bone marrow biopsy or a bone marrow aspiration.
The treatment of ES is likely to include a combination of:
The choice of therapies, timing and duration will be chosen by a team of oncologists 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:
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.
Visit the trusted websites below to learn more about ES.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
Riley at IU Health is a member of the Children's Oncology Group Phase 1 & Pilot Consortium, an organization that is dedicated to researching and finding new treatments for childhood cancer. The National Cancer Institute also sponsors some of the cancer research we do at Riley at IU Health. Ask your child's doctor for more information about current research studies or clinical trials on ES or visit ClinicalTrials.gov. Please note: Not all clinical trials may be applicable to pediatric patients or your child's type or stage of cancer. Please consult your child's doctor with questions regarding available trials.
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