Supratentorial pediatric cortical ependymomas: a comprehensive retrospective study

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.