Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: Results of the German prospective trials HIT 88/89 and HIT 91

Purpose: To evaluate the outcome in children with anaplastic ependymomas af ter surgery, irradiation, and chemotherapy; and to identify prognostic fact ors for survival. Methods and Materials: Fifty-five children (n = 27 girls, 28 boys; median a ge at diagnosis, 6.2 years) with newly diagnosed anaplastic ependymomas wer e treated in the multicenter, prospective trials HIT 88/89 and HIT 91, Macr oscopic complete resection was achieved in 28 patients; 27 patients underwe nt incomplete resection. All patients received chemotherapy before (n = 40) or after irradiation (n = 15). The irradiation volume encompassed either t he neuraxis followed by a boost to the primary tumor site (n = 40) or the t umor region only (n = 13), No radiotherapy was administered in two patients . Results: Median follow-up was 38 months. The overall survival rate at 3 yea rs after surgery was 75,6%, Disease progression occurred in 25 children wit h local progression occurring in 20, The median time to disease progression was 45 months. The only significant prognostic factor was the extent of re section (estimated progression-free survival [EPFS] after 3 years was 83.3% after complete resection and 38.5% after incomplete resection) and the pre sence of metastases at the time of diagnosis (0% vs. 65.8% 3-year EPFS in l ocalized tumors), Age, sex, tumor site, mode of chemotherapy, and irradiati on volume did not influence survival. Conclusions: Treatment centers should be meticulous about surgery and diagn ostic workup, Because the primary tumor region is the predominant site of f ailure it is important to intensify local treatment. Dose escalation by hyp erfractionation or stereotactic radiotherapy might be a promising approach in macroscopically residual disease. The role of adjuvant chemotherapy requ ires further study. (C) 2000 Elsevier Science Inc.