Ependymoma of the Spinal Cord in Children: A Retrospective French Study

Abstract

Ependymoma is the most frequent spinal tumor in adults but it is rather uncommon in children. The aim of the present study was to retrospectively summarize the clinical and therapeutic experience in the treatment of pediatric spinal ependymomas in France. In the present retrospective multicenter study, data from patients aged <18 years who had been treated from 2000 to 2010 for spinal ependymomas were collected. Epidemiologic and tumor- and treatment-related data were analyzed. The prognostic factors for progression-free survival (PFS) were assessed. We identified 28 patients (22 males, 6 females). Their median age at surgery was 13.67 years (range, 0.7-17.6). Initial gross total resection (GTR) was achieved in 22 children and subtotal resection (STR) in 6. Histologically, 15 tumors were myxopapillary ependymomas and 11 were grade II and 2 grade III ependymomas. Adjuvant initial radiotherapy (RT) was performed in 6 patients. The median follow-up period was of 40 months (range, 2.3-127.5). The 5-year PFS rate was 51% (95% confidence interval, 26.3%-71.2%), and the overall survival rate was 100%. On univariate analysis, only GTR had a significant influence on PFS (P= 0.0013). A subgroup analysis showed a benefit of RT delivered to patients with GTR; however, RT failed to prevent relapse in the group with initial STR. Our data suggest that initial adjuvant RT might improve PFS after GTR but will not prevent relapse in patients with STR. Further studies are needed to define more specific treatments for the latter group.