Influence of tumor grade on time to progression after irradiation for localized ependymoma in children

Abstract

Purpose: To investigate the influence of histologic grade on progression-free survival (PFS) after irradiation (RT) for pediatric patients with localized ependymoma. Methods and Materials: Fifty patients with localized ependymoma (median age 3.6 years, range 1–18 years at the time of RT) were treated with RT between December 1982 and June 1999. Anaplastic features were identified in 14 of 50 patients. The extent of resection was characterized as gross-total in 36 patients, near-total in 5, and subtotal in 9. The median dose to the primary site was 54 Gy. Of the 50 patients, 23 received pre-RT chemotherapy. Results: Thirty-nine patients were alive at a median follow-up of 46 months (range 21–214) from diagnosis. Thirty-four patients remained progression free at a median follow-up of 35 months (range 13–183) after the initiation of RT. Progression occurred in 16 patients (12 local and 4 local and distant), with a median time to failure of 21.2 months (range 4.6–65.0). The tumor grade significantly influenced the PFS after RT ( p < 0.0005). The estimated 3-year PFS rate was 28% ± 14% for patients with anaplastic ependymoma compared with 84% ± 8% for patients with differentiated ependymoma. These results remained significant when corrected for age at diagnosis (<3 years), pre-RT chemotherapy, and extent of resection. Patients who received pre-RT chemotherapy had an inferior 3-year PFS estimate after RT (49 ± 12%) compared with those who did not (84% ± 10%; p = 0.056). Anaplastic ependymoma was found more frequently in the supratentorial brain ( p = 0.002). Six of 12 patients with supratentorial tumor developed recurrence; recurrence was restricted to patients with anaplastic ependymoma. Conclusion: Tumor grade influences outcome for patients with ependymoma independent of other factors and should be considered in the design and analysis of prospective trials involving pediatric patients treated with RT. Chemotherapy before RT influences the PFS and overall survival after RT. The effect is more pronounced when progression occurs during chemotherapy.