Department of Neurosurgery, West China Hospital of Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu, 610041, Sichuan, China.
Neurosurg Rev. 2021 Jun;44(3):1543-1551. doi: 10.1007/s10143-020-01336-w. Epub 2020 Jun 30.
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.
儿童脑室外皮瘤（CE）较为罕见，其临床特征和最佳治疗方案仍不明确。本研究旨在通过机构系列研究和文献复习，阐明儿童 CE 的临床特征和预后。本研究纳入了来自我们科室的 13 例 CE 患儿。此外，通过对英文同行评议文献的检索，共纳入了 43 例 CE 患儿。对这些患儿的临床资料、治疗方法和预后进行了回顾性分析。我们的机构系列研究包括 9 例男性和 4 例女性。文献复习共纳入 56 例儿童 CE 病例（包括我们的病例）进行进一步分析。其中，额叶（n=19，41.3%）是最常见的发病部位，且大多数肿瘤位于右侧大脑半球（n=27，58.7%）。术前最常见的症状是癫痫发作（n=23，41.1%）。30 例患儿（n=30，53.6%）为世界卫生组织（WHO）分级 II 级。我们连续 5 例患儿进行 C11orf95-RELA 融合筛查，所有患儿（100%）均为 RELA 融合阳性。14 例（26.4%）患儿肿瘤复发，4 例（7.5%）患儿在随访期间死亡。多变量生存分析显示，手术切除范围是 PFS 的唯一预后因素，完全切除肿瘤的患儿（P=0.037，HR 3.682，95%CI 1.082-13.79）PFS 更长。此外，Kaplan-Meier 生存分析的对数秩检验显示，手术切除范围（P=0.007）是 OS 的唯一预后因素。儿童 CE 较为罕见，常见于额叶和右侧大脑半球。癫痫发作是最常见的症状。CE 可能存在更高的 RELA 融合率，但预后良好。低级别组织学表现为非典型性的发生率较低。肿瘤全切除与更长的 PFS 和 OS 显著相关。由于肿瘤可能进展，需要仔细随访。
