SIOP Study of Combined Modality Treatment in Childhood Ependymoma

OBJECTIVES: Determine the event free and overall survival of children with incompletely resected localized ependymoma when treated with adjuvant cyclophosphamide, etoposide, and vincristine followed by radiotherapy. Determine the response rate in these patients to this regimen. OUTLINE: This is a multicenter study. Patients undergo surgery to remove as much of tumor as possible. Patients with residual disease proceed to chemotherapy, while those with no residual disease proceed directly to radiotherapy. Chemotherapy begins within 3 weeks of surgery and consists of vincristine IV on days 1, 8, and 15, cyclophosphamide IV over 3 hours on day 1, and etoposide IV over 4 hours on days 1-3. Treatment repeats every 4 weeks for up to 4 courses. Patients who progress after 2 courses proceed to radiotherapy. If residual disease is still present at completion of chemotherapy, second look surgery is recommended. Patients undergo radiotherapy daily for 6 weeks beginning after complete resection within 4 weeks of surgery, within 3 weeks of completion of chemotherapy, or within 4 weeks of second look surgery. Patients are followed at 6 weeks after radiotherapy, every 2 months for 1 year, every 4 months for 2 years, every 6 months for 2 years, and then annually for 5 years. PROJECTED ACCRUAL: A total of 65 patients will be accrued for this study within 2-3 years.