Current management in the treatment of intramedullary ependymomas in children

Abstract Purpose: Current management of pediatric intramedullary ependymoma is extrapolated from adult series since large studies in children are not available. This has led us to share our experience with this rare tumor and compare it to the literature; in order to review and highlight important aspects of current management and point out inconsistencies.Methods: A retrospective analysis of patients with intramedullary ependymoma managed at our institution between 2004 and 2021.Results: During the study period 5 patients were treated for intramedullary ependymoma. Cases of myxopapillary ependymoma were excluded. The mean age of our cohort was 11,2 years. We identified 4 cases of grade II ependymoma and 1 case of grade III ependymoma. Gross tumor removal (GTR) was achieved in two patients (40%) of patients. One patient was treated with radiotherapy for recurrence and two patients received chemotherapy. There were no cases of recurrence among patients treated with GTR, but in all patients treated with STR. 80% of patients either improved or stayed stable neurologically. During follow-up (mean 73 months) 2 patients died of disease.Conclusion: GTR and tumor grade remain the key prognostic factor of long-term tumor-free survival. Many questions prevail with regards to outcomes, correct use of adjuvant therapy, and prognostic factors.