Spinal cord ependymomas in children and adolescents

Abstract

Spinal cord ependymomas are very rare among children and adolescents. Due to their rarity, our current knowledge of these tumors is based on case reports and few retrospective case series. The present review summarizes the currently available literature on childhood spinal cord ependymomas. Although overall survival rates are favorable, relapse incidence is high, particularly in myxopapillary ependymomas. Since long-term follow-up data are provided in a limited number of studies only, the true relapse incidence is unknown. Maximal safe radical surgery is the backbone of treatment for children with spinal cord ependymomas, but the impact of adjuvant treatment on progression and survival is still unclear. Presently, the decision to initiate non-surgical treatment depends primarily on the WHO grade of the tumor and the extent of resection. In terms of the known side effects, early radiotherapy should be avoided in children with WHO grade II spinal cord ependymomas irrespective of the extent of resection but is indicated in anaplastic spinal cord ependymomas both after complete and incomplete resection. The high relapse incidence in myxopapillary ependymomas argue for the use of early radiotherapy, but its definitive impact on progression has to be proven in larger series. Close surveillance is important due to the high recurrence rate in all patients with spinal cord ependymomas. Prospective collection of both clinical and molecular data from a greater number of patients with spinal cord ependymomas within an international collaboration is the prerequisite to establish standardized management guidelines for these rare CNS tumors.