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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.
Insights
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.
Abstract:
Pediatric cortical ependymomas (CEs) are rare; the clinical features and optimal treatment remain ill-defined. We aimed to clarify the clinical characteristics and outcome of pediatric CEs based on institutional series and literature review. Thirteen children with CEs from our department were included in the present study. Furthermore, a search of English language peer-reviewed articles yielded 43 patients with CEs. The clinical data, treatment, and outcome were retrospectively reviewed and statistically analyzed. Our institutional series consisted of nine males and four females. The literature review yielded 56 pediatric CE cases (including ours) for further analysis. Of these 56 cases, frontal lobe (n = 19, 41.3%) was the most common location and most of the tumors were located in the right hemisphere (n = 27, 58.7%). Seizures (n = 23, 41.1%) were the most frequent preoperative symptoms. Thirty patients (n = 30, 53.6%) were WHO grade II. Five continuous patients in our series screened for C11orf95-RELA fusion and all the patients (100%) were RELA fusion positive. Fourteen (26.4%) patients experienced tumor recurrence and 4 (7.5%) patients died during the follow-up. Multivariate survival analysis depicted extent of surgery resection was the only prognostic factor for PFS and patient with gross total resection (P = 0.037, HR 3.682, 95% CI 1.082-13.79) had longer PFS. Furthermore, Log-rank testing for Kaplan-Meier survival analysis showed the extent of surgery resection (P = 0.007) was the only prognostic factor for OS. Pediatric CEs are rare, commonly seen in frontal lobe and right hemisphere. Seizures are the most common symptoms. They may have higher rate of RELA fusions, but favorable outcome. A low incidence of anaplastic histology has been depicted. Gross total resection is significantly associated with longer PFS and OS. Careful follow-up is necessary because the tumors may progress.
