What is Ewing Sarcoma?

Ewing Sarcoma, named after James R. Ewing, who described the first cases in 1921, is an aggressive cancer that affects bones or nearby soft tissue. Cancer occurs when healthy cells change and grow out of control. Ewing Sarcoma tumors can occur in any bone in the body but most often occur in the long bones of the legs or arms, as well as the ribs, spine, and pelvis. If the cancer metastasizes, or spreads, it usually goes to the lungs, other bones, and the bone marrow. Approximately 250 cases of Ewing Sarcoma are diagnosed each year in the United States. Most cases involve kids and young adults while more males than females suffer from the disease.

Ewing Sarcoma Treatment

The treatment for Ewing Sarcoma typically consists of chemotherapy combined with surgery and/or radiation.

The primary chemotherapy regimen is known as VDC/IE. This chemo alternates between two combinations of drugs given every two to three weeks. The first drug combination includes Vincristine, Doxorubicin, and Cyclophosphamide. The second set of chemo agents includes a combination of Ifosfamide and Etoposide. Cyclophosphamide was approved for use in 1959, followed by Vincristine (1963), Doxorubicin (1974), Etoposide (1983) and finally Ifosfamide which was approved in 1987.

Surgery and/or radiation is often combined with Ewing Sarcoma chemotherapy. Tumors in the arms or leg can be removed while saving the limb. This surgery, called limb salvage, replaces the removed bone with a bone graft (a piece of bone from elsewhere in the body or another person) or an internal prosthesis (a bone shaped piece of metal of other material). Amputation, which is the removal of part or all of the limb, is also seen in Ewing Sarcoma treatment. Tumors and affected bone that can’t be removed surgically are often treated with radiation. In many cases, a combination of surgery and radiation is chosen to improve the long-term prognosis.

The overall five-year survival rate for a patient with Ewing Sarcoma is between 60-70%. The last major advance in treatment came twenty years ago with clinical trials demonstrating that five drugs were better than three and interval compression (every two- week cycles) were superior to conventional timing (every three-week cycles).

Recurrent Ewing Sarcoma

Standard care for Ewing Sarcoma patients revolves around VDC/IE chemotherapy and surgery/radiation. However, if this frontline treatment in unsuccessful and the cancer returns, called recurrent Ewing Sarcoma, there is no agreed upon treatment protocol. Oncologists are left to try alternate chemotherapy agents in hopes to slowdown or stop the rapidly dividing Ewing Sarcoma cells. Depending on the location of the new tumor, surgeons are forced to remove more of the patient’s original anatomy or use higher doses of radiation. Finally, the recurrent Ewing Sarcoma patient is faced with months and sometimes years of additional chemotherapy, surgeries, medical care, and a much lower survival rate.