Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life

Abstract

Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes. A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques. A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2-100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710). Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.