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Supratentorial pediatric cortical ependymomas: a comprehensive retrospective study.
Qiguang Wang1, Jian Cheng1, Si Zhang1
1Department of Neurosurgery, West China Hospital of Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu, 610041, Sichuan, China.
Neurosurgical Review
|July 2, 2020
Summary
Pediatric cortical ependymomas (CEs) are rare brain tumors. Complete surgical removal is key for longer progression-free survival and overall survival in children with CEs.
Area of Science:
- Neuro-oncology
- Pediatric neurosurgery
- Molecular diagnostics in oncology
Background:
- Pediatric cortical ependymomas (CEs) are rare central nervous system tumors with poorly defined clinical characteristics and treatment strategies.
- Understanding the clinical presentation, molecular features, and prognostic factors is crucial for improving outcomes in affected children.
Purpose of the Study:
- To elucidate the clinical features, treatment modalities, and outcomes of pediatric cortical ependymomas.
- To identify prognostic factors influencing progression-free survival (PFS) and overall survival (OS) in pediatric CEs.
- To investigate the prevalence of C11orf95-RELA fusions in pediatric CEs.
Main Methods:
- Retrospective analysis of a cohort of 13 pediatric patients with CEs from an institutional series.
- Comprehensive literature review and statistical analysis of 43 additional pediatric CE cases, totaling 56 cases.
- Evaluation of clinical data, treatment records, and outcomes, including PFS and OS. C11orf95-RELA fusion screening was performed on a subset of patients.
Main Results:
- Pediatric CEs most commonly occurred in the frontal lobe (41.3%) and right hemisphere (58.7%). Seizures were the most frequent presenting symptom (41.1%).
- WHO grade II was observed in 53.6% of cases. All tested patients (100%) exhibited C11orf95-RELA fusion.
- Gross total resection was significantly associated with longer PFS (P=0.037) and OS (P=0.007), identified as the sole independent prognostic factor.
Conclusions:
- Pediatric CEs are characterized by frontal lobe predominance, seizures as a common symptom, and a high rate of RELA fusions, yet demonstrate a generally favorable outcome.
- Gross total resection is a critical determinant for improved PFS and OS in pediatric cortical ependymomas.
- Despite a low incidence of anaplastic histology, careful long-term follow-up is essential due to the potential for tumor progression.
