Improved Survival for Those With Ewing Sarcoma
Key Words: chemotherapy, etoposide, Ewing sarcoma, ifosfamide. (Definitions of many terms related to cancer can be found in the Cancer.gov Dictionary.)
Addition of the drugs ifosfamide and etoposide to standard chemotherapy for Ewing sarcoma -- a rare but highly malignant bone tumor affecting children and young adults -- significantly improved five-year survival in patients whose disease had not spread to other organs. The study results were reported in the February 20, 2003, issue of the New England Journal of Medicine (see the journal abstract of the study.)
Previously, standard therapy for Ewing sarcoma involved surgery, radiation therapy, or both, plus chemotherapy with the drugs doxorubicin, vincristine, cyclophosphamide, and dactinomycin. In the current study, 518 patients were assigned at random to one of two groups. One group received the standard chemotherapy regimen. The second group received experimental therapy in which the four standard drugs were alternated with ifosfamide and etoposide. Of the 518 patients, 120 had advanced (metastatic) disease; the remainder had localized disease (not spread to other organs).
Five-year survival
Among the patients with localized disease, 69 percent of those who received the experimental therapy were alive, with no recurrence of their disease, after five years. By contrast, 54 percent of those who received standard therapy were alive and disease-free after five years. Overall survival at five years was 72 percent in the experimental therapy group, compared with 61 percent in the group treated with standard therapy.
However, among patients whose disease had spread to other organs, those receiving the experimental therapy fared no better than those treated with the standard drug regimen. After five years, 22 percent of patients in both groups were alive and disease-free. Overall survival at five years for patients with advanced disease was 34 percent in the experimental-therapy group and 35 percent in the standard-therapy group, a difference that was statistically insignificant (that is, it could have occurred by chance).
More infections, more hospital days
Patients who received the experimental therapy suffered more infections and spent more days in the hospital than those who received standard therapy. Regardless of which treatment they received, patients aged 17 and under and those with smaller tumors had better survival rates than patients who were aged 18 and over or who had large tumors.
In the United States each year, about 200 children and adolescents are diagnosed with Ewing sarcoma or one of its close cousins. These tumors are most common in the teenage years. Most occur in the long bones of the legs or arms.
The research was conducted by the Children’s Cancer Group and the Pediatric Oncology Group, with a research team led by Holcombe E. Grier, M.D. These two groups have since merged to form the Children's Oncology Group (COG), which is supported by the National Cancer Institute to conduct clinical trials around the world devoted to children and adolescents with cancer.
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