• Jock C Lillard, Garrett T Venable, Nickalus R Khan, Ruth G Tatevossian, James Dalton, Brandy N Vaughn, and Paul Klimo.
• College of Medicine, University of Tennessee Health Science Center, Memphis, Tennessee.
• Neurosurgery. 2019 Jul 1; 85 (1): 41-49.

BackgroundPediatric supratentorial ependymomas (SEs) have distinct molecular and behavioral differences from their infratentorial counterparts.ObjectiveTo present our experience with pediatric SEs over a 24-yr period.MethodsClinical, operative, and radiographic information was abstracted retrospectively. Our primary outcomes were progression-free survival (PFS) and overall survival (OS). Detection of C11orf95-RELA rearrangement was performed using interphase fluorescence in situ hybridization (iFISH).ResultsSeventy-three patients were identified (41 female, 32 male); median age was 6.7 yrs (range, 1 mo-18.8 yr); median follow-up was 8.3 yrs (range, 2.0-26.3). Fifty-eight (79.5%) of 73 patients underwent gross total resection (GTR); no patient with subtotal resection had greater than 1 cm3 of residual tumor; 42 patients (57.5%) experienced subsequent disease progression with 17 patients ultimately dying of their disease. Median PFS was 3.7 yrs. Molecular analysis was available for 51 patients (70%). On bivariate analysis, PFS and OS were not statistically affected by age, tumor grade, or extent of resection, although there was a clinically significant trend for the latter in favor of aggressive resection on PFS (P = .061). Children with RELA fusion had significantly higher PFS (P = .013) than those without, although there was no difference in OS when compared with those with no C11orf95-RELA fusion or C11orf95 gene rearrangement alone.ConclusionIn our series, GTR may be associated with better PFS, but did not impact OS. Surprisingly, RELA fusion was not found to be a negative prognostic factor, raising the possibility that the deleterious effects may be overcome by aggressive resection.Copyright © 2018 by the Congress of Neurological Surgeons.

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