Ewing Sarcoma
Ewing sarcoma is the second most common kind of cancerous bone tumor that affects children and young adults.
What Is Ewing Sarcoma?
Ewing sarcoma is a form of solid tumor cancer that usually starts in the bone. It typically occurs in the long bones of the legs or arms or in the pelvis, chest wall, spine or skull. It is the second most common tumor of the bone. Ewing sarcoma can also begin in the soft tissues. The lungs and other bones are the most common locations for Ewing sarcoma to spread (metastasize).
These are called the Ewing family of tumors (EFTS) and include:
- Ewing sarcoma
- Atypical Ewing sarcoma
- Peripheral primitive neuroectodermal (PNET) tumor of the bone
Ewing sarcoma most often affects children and young adults between 10 and 25 years old.
What Are the Symptoms of Ewing Sarcoma?
The most common symptom is bone pain that keeps getting worse. You might feel a lump near the surface of the skin. It might feel warm and soft to the touch. Other symptoms include:
- Swelling
- Redness
- Fever
- Tiredness (fatigue)
- Weight loss
- Soreness (tenderness) of tissues over the affected bone
- Chest pain and shortness of breath for tumors in the ribs
- Back pain for tumors in the pelvis
How Is Ewing Sarcoma Diagnosed?
An X-ray of the bone may show possible Ewing sarcoma, but a biopsy is performed to confirm a diagnosis. A biopsy (a sample of the tissue of the affected bone) will be taken by the surgeon in the operating room or in Interventional Radiology initially. This depends on where the tumor is located. The tissue sample will be examined under a microscope by a pathologist to determine if it is Ewing sarcoma.
Because Ewing sarcoma can spread to other parts of the body, a careful look for this spread is done before any further surgery or treatment. A chest computed tomography (CT) scan is necessary to observe for spread to the lungs.
A positron emission tomography (PET) whole body bone scan screens for other lesions. An MRI scan should be performed before the bleeding and edema from a biopsy have occurred. For extremity tumors, MRI should include at least one joint above and below the tumor to evaluate for skip lesions. A bone marrow biopsy is crucial to determine if there is spread of the disease.
How Is Ewing Sarcoma Treated?
Treatment for Ewing sarcoma involves surgery for removal of tumor, chemotherapy, radiation or a combination of therapies.
- Chemotherapy. Chemotherapy uses drugs to kill cancer cells. The type, amount and how often chemotherapy is completed will be decided by the oncologist (cancer doctor). In addition to killing cancer cells, chemotherapy drugs affect healthy cells, causing various side effects. The goal of chemotherapy is to reduce the spread of the tumor or eradicate it completely. Chemotherapy is given over a period of 8 to 12 months. Your child will need to be admitted to the hospital for 3-6 days or more each time.
- Surgery. Surgical treatment usually happens after several cycles of chemotherapy. The type of surgery depends on where the tumor is located. Your child’s care team will determine the best surgical approach for your child.
- Radiation therapy is sometimes used to shrink the tumor before surgery or destroy any remaining tumor cells after surgery. These are among other radiation therapies. Options include high-energy X-rays, injection of a radioactive substance into the tumor, or proton therapy. Proton therapy uses protons (positively charged particles) instead of the X-rays.
Your child will have follow-up appointments with an oncologist and oncologic orthopedist. Every few months there will be more X-rays, chest CT or PET scans to check for signs of the tumor getting smaller or returning. Blood tests will be done to check for side effects of therapy.
