Jason Yustein, M.D., Ph.D., a St. Baldrick’s Foundation Board Member and Scientific Program Committee Member, at Emory University School of Medicine, Georgia, explains Ewing sarcoma symptoms, treatment options, and research opportunities.
What is Ewing sarcoma?
Ewing sarcoma is a type of childhood cancer that is most frequently found in children and adolescents between the ages of 10 and 20 years old.
Ewing sarcoma — the second most common bone cancer after osteosarcoma — often originates in the long, large bones of the body, including the hip, thigh, shin, chest, and arm bones.
Besides originating from the bone, sometimes this tumor can form just outside the bone in the soft tissue.
The cancer can also move to other sites including the lungs, other bones or soft tissue areas, and bone marrow. This movement, known as metastasis, often makes the disease more difficult to treat, resulting in significantly lower survival rates. Approximately 1 in 4 children with Ewing sarcoma will have metastatic disease at the time of first diagnosis.
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What are the signs and symptoms of Ewing sarcoma?
- Swelling of the bone that can cause persistent pain
- Bone fracture that can happen all of a sudden or after minor bumps
- Pain or difficulty breathing (if the tumor affects the ribs)
- A limp (if the tumor affects the leg)
How is Ewing sarcoma diagnosed?
To diagnose Ewing sarcoma, a pediatric oncologist will take a biopsy of the tumor and bring those tumor cells to the lab. Looking at the cells under a microscope allows physicians to determine the specific tumor type.
The patient will receive x-rays and an MRI of the tumor area, as well as a CT scan of the chest to find out whether the cancer has spread to the lungs. A radioisotope scan of all the bones in the body will confirm whether there is disease in any other bones.
The patient will also undergo a bone marrow biopsy to determine whether cancer is present in the marrow or blood.
How is Ewing sarcoma treated? What are the side effects of treatment?
Ewing sarcoma can be very painful and require a large amount of medications. Children with this type of cancer may need surgery and sometimes radiation therapy in order to treat them and get rid of the tumor.
Unfortunately, the survival outcome for Ewing sarcoma patients is less than most other types of pediatric cancers. These patients also have to deal with tremendous side effects due to high-dose chemotherapy and the debilitating surgeries that are often part of treatment for Ewing sarcoma.
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How is research helping kids with Ewing sarcoma?
Research is dedicated toward understanding how these tumors live and grow, so we can create better ways to kill the tumors and improve patient outcomes.
For instance, genetic sequencing is helping researchers gather data on mutations associated with Ewing sarcoma, while other research has revealed new insights into the role of a certain protein, called EWS-FLI1, which is seen in most of these tumors.
Additionally, studies have found that Ewing sarcoma cells are vulnerable to specific targeted therapies, especially a certain class of inhibitors – drugs that block the actions of its target protein – called PARP inhibitors. Developments are promising, especially when it comes to using these inhibitors in combination with other targeted therapies.
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