Each year in the United States, approximately 300 to 400 children, adolescents, and young adults up to age 40 are diagnosed with Ewing sarcoma.

The disease was first described here at Memorial Sloan Kettering in 1921 by pathologist James Ewing. It is a tumor that involves the bone as well as the soft tissue such as that in the pelvis or chest wall. It can develop in the middle area of bones — in the femur, pelvic bone, ribs, upper arm, and thigh. Patients most commonly experience pain and swelling in the affected area.

Diagnosis

When our doctors suspect that a child has Ewing sarcoma, the diagnosis is confirmed using x-rays, CT scans, MRI, and a biopsy of the affected tissue. Because Ewing sarcoma can spread widely throughout the body, our experts often examine the bone marrow and perform imaging studies such as chest CT scans, PET scans, and bone scans to determine how far the tumor has spread.

Treatment

Our experts treat patients with Ewing sarcoma using chemotherapy to shrink the tumor and to prevent new tumors from forming. Standard chemotherapy medications used include vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide.

We also perform surgery to remove the tumor, and depending on where the tumor is located, radiation therapy may also be used to treat Ewing sarcoma. For patients with Ewing sarcoma at a high risk of recurrence, we are developing a new clinical trial of the drugs irinotecan and temozolomide.

If a child’s disease returns after treatment, possible additional treatments could include a second surgery and/or radiation therapy. Your child may also be eligible for innovative therapies through clinical trials.

One of our clinical trials for newly diagnosed patients is building on our success in treating Ewing sarcoma that has returned after conventional therapy. In the past, we have shown that patients with recurrent disease responded well to the combination of two new drugs: irinotecan and temozolomide.(1) We have added these agents to the others that have been traditionally used to treat newly diagnosed Ewing sarcoma in an effort to improve the chances of successfully treating these patients.