Introduction: Recent molecular analyses support a behavioral and clinical distinction between supratentorial and infratentorial ependymomas, with supratentorial tumors, in general, having a more favorable prognosis. The goal of this study was todescribe our experience managing supratentorial ependymoma in children.
Methods: A prospectively maintained neurooncology database was queried to identify cases ofsupratentorial ependymoma treated atSt Jude Children’s Research Hospital (SJCRH) and LeBonheur from 1990 through December 31, 2014. Clinical, operative, and radiographic information were reviewed. Outcome measures, determined by review of clinic notes and subsequent imaging, included extent of resection, progression free survival (PFS), overall survival (OS), recurrence of disease and method of post-failure treatment, seizures, requirement of anti-seizure medications, hydrocephalus requiring shunt placement, and death.Detection ofthe C11or95-RELA fusion or rearrangement was performed using iFISH in those patients whose tumor tissue was still available.
Results: Seventy-four cases (42% male) of supratentorial ependymoma were identified. Median age at diagnosis was 6.8 years. In total, 59 (79.7%) of74 patients underwent gross total resection (GTR). Overall survival was a median of 7.4years. Fifteen patients died of disease.Progression free survival was a median of 3.6 years. Eighty percent of those children whose initial resection was subtotal developed recurrence compared to 50.8% in those who had a GTR.For those patients that recurred, all 42 received further treatment, including further resection, chemotherapy and radiation. Molecular analysis was available for 52 patients (70.3%). Age less than 3, gross total resection, and tumor grade were not associated with improved PFS or OS. Absence of C11-or95-RLEA fusion was associated with a significantly worse PFS, although OS was similar.
Conclusions: Supratentorial ependymoma often have a more favorable prognosis compared toinfratentorial variants. Like many pediatric tumors, supratentorial ependymomas can bestratified based on clinical, surgical and possibly molecular variables.
Patient Care: It will provide additional insight into prognostic indicators and guide future treatment modalities
Learning Objectives: 1. Understanding ependymomas
2. Appreciating the complexity of these tumors based on different locations and molecular subtypes
3. Analyzing prognostic indicators to guide future treatment