Ewing’s Sarcoma
What is it?
Ewing’s sarcoma is a disease in which cancer cells in the form of a tumor are found in and/or around a bone. It is the second most common type of bone cancer in children and adolescents and accounts for about 3 percent of all childhood cancers. Ewing’s sarcoma most often occurs in school age children, adolescents and young adults.
The most common tumor locations include the arm and leg bones, the pelvic bone and the chest wall (ribs). However, Ewing’s sarcoma can occur in any bone of the body. The muscles and soft tissues around the tumor can also be affected to varying degrees.
Some tumors are localized, meaning the cancer cells have not spread beyond the bone and nearby tissues to other parts of the body. Other tumors are metastasis, meaning the cancer cells spread to other parts of the body. The most common places for the cancer cells to spread are to the lungs and/or other bones.
Why come to The Children’s Hospital for treatment of this condition?
The Children’s Hospital sees and treats only children, adolescents and young adults, making us experts in caring for young people. The Orthopedic Oncology team and the Center for Cancer and Blood Disorders at Children’s will ensure that your child receives the most accurate diagnosis and the best treatment possible. As your child is likely still growing, it is important to choose a treatment team that specializes in pediatric bone tumors who will take this and other factors into consideration.
The bone and soft tissue cancer team at The Children’s Hospital consists of a multidisciplinary team of medical professionals that will provide your child with the most compassionate and quality care that can be delivered. This team includes a doctor who specializes in bone tumor surgery (orthopedic oncologist); a cancer specialist (pediatric oncologist), who is in charge of the chemotherapy; and a radiation oncologist, who is the doctor in charge of radiation therapy if it is needed. All of your child’s treatment will be done on the campus of The Children’s Hospital.
Additional members of the team include a nurse practitioner, nurses who specialize in orthopedic oncology, physical therapists, child life specialists, occupational therapists and various others who are trained to give your child the excellent care that they need and deserve.
What are the signs and symptoms?
The most common symptoms of Ewing’s sarcoma include:
- Pain (can occur with activity or at rest)
- Swelling or presence of a palpable mass (bump)
- An unexplained fever with no symptoms
- Unexplained weight loss
- Poor appetite
- Fatigue
It is not uncommon for the child to experience a minor injury or have a limp which brings attention to the tumor. Symptoms can also appear to be similar to symptoms of an infection.
If your child or teen has these symptoms it is important to see a doctor immediately - early treatment of Ewing ’s Sarcoma is more likely to be successful.
How do you diagnose it?
To ensure your child receives the most accurate diagnosis and the best treatment possible, it is important to choose a doctor who specializes in pediatric bone tumors.
A bone tumor specialist will begin by getting a detailed medical history, family history, and perform a comprehensive physical exam. Radiographic studies are usually performed - these may include magnetic resource imaging (MRI), bone scan or a CAT scan of the lungs. The ultimate diagnosis is always based on a biopsy of the tumor with a diagnosis coming from pathology and examination of the biopsy specimen. Often times, a preliminary diagnosis is available on the day of biopsy; however, it may take up to a week to get a permanent diagnosis depending on what additional studies need to be done.
After the diagnosis of Ewing’s sarcoma is made, your child’s bone tumor specialist will evaluate the extent of the disease by performing several different tests. This is known as staging. These tests may include a bone scan, a PET scan, a CAT scan or an MRI. The doctor will also order blood tests. The studies are done to look for any evidence that the tumor has spread from the original site and are necessary in order to provide the most definitive treatment for the disease.
How do we treat it?
Ewing’s sarcoma is typically treated with chemotherapy, surgery and radiation therapy. Treatment recommendations may include a combination of the following.
Chemotherapy
Chemotherapy is medicine initially given prior to surgery to help shrink the size of the tumor and other cancer cells. It is administered through a special type of IV, known as a port, that goes into a large vein that is put into the child's chest surgically. The medicine goes directly into the child’s vein through a catheter (tube) in order to enter the bloodstream and work to destroy the cancer cells throughout the body.
Radiation Therapy
Radiation therapy is sometimes used by itself or in combination with chemotherapy and surgery to treat the tumor.
Surgery
The Ewing’s sarcoma tumor will be removed by surgery. The kind of surgery that your child will have depends on the size of the tumor, where the tumor is located and if the cancer cells have spread. Surgical options may include:
- Limb-Sparing Surgery: This type of surgery removes the tumor and the tissues surrounding it that contain cancer cells, while leaving the surrounding muscles, nerves and blood vessels. A bone graft or metal implant will replace the bone and soft tissues that have been removed, thereby preserving the patient’s extremity. After surgery, activities are significantly restricted until the patient has sufficiently healed. During this time your child will receive physical therapy and may have crutches, a walker or a wheelchair. Over time, the child can gradually return to activities.
- Amputation : Sometimes the tumor has to be taken out with removal of the affected extremity. This may be done because the tumor is too large or has a high risk of recurring. Children are typically very good at using and adapting to an artificial limb (prosthetics). Children who have amputations can live happy and normal lives - many people with artificial limbs are quite active and participate in sports.
Who gets it, and can it be prevented?
Ewing’s sarcoma most often occurs in school age children (ages 8-12), adolescents and young adults and affects both boys and girls. There is no known cause or methods of prevention for Ewing’s sarcoma at this time.
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