Department of Neurological Surgery, School of Medicine, Wakayama Medical University.
School of Health and Nursing Science, Wakayama Medical University.
Neurol Med Chir (Tokyo). 2022 Jul 15;62(7):322-327. doi: 10.2176/jns-nmc.2022-0027. Epub 2022 May 10.
To assess the clinicopathological features and prognostic factors of pediatric intracranial ependymomas and to explore the current diagnostic practice, we analyzed clinical data from the Brain Tumor Registry of Japan (BTRJ). Data of fifty children under 18 years of age diagnosed with intracranial ependymoma were extracted from the BTRJ database. Cases were reviewed for overall survival (OS) and progression-free survival (PFS), with attention to gender, preoperative Karnofsky performance status score, location of the tumor, the extent of resection, World Health Organization (WHO) histopathological grading, and adjuvant therapy. The median age at diagnosis was 6.1 years, ranging from 7 months to 17.6 years. Based on the WHO histopathological grading, 27 patients were classified under grade 2 (54%) and 23 patients were classified under grade 3 (46%). Gross total resection (GTR) was achieved in 30 patients (60%). The median follow-up time was 65 months. Five-year PFS and OS were 47.2 ± 7.3% and 73.3 ± 6.7%, respectively. GTR was associated with longer OS (P = 0.02). The histopathological grading was not an independent prognostic factor for the OS. Mitosis and microvascular proliferation were higher among patients with grade 3 than in those with grade 2, which aided in deciding the WHO grade. This nationwide study revealed the characteristics and outcomes of patients with childhood ependymomas. GTR was the factor most consistently associated with improved survival. In contrast, the histopathological grading in this cohort was not a significant prognostic factor. More reproducible and practical criteria for the diagnosis of intracranial ependymomas should be further pursued in future studies.
为了评估儿童颅内室管膜瘤的临床病理特征和预后因素，并探讨当前的诊断实践，我们分析了日本脑瘤登记处（BTRJ）的临床数据。从 BTRJ 数据库中提取了 50 名 18 岁以下诊断为颅内室管膜瘤的儿童病例。对总生存期（OS）和无进展生存期（PFS）进行了回顾性分析，重点关注性别、术前卡诺夫斯基表现状态评分、肿瘤位置、切除程度、世界卫生组织（WHO）组织病理学分级和辅助治疗。诊断时的中位年龄为 6.1 岁，范围为 7 个月至 17.6 岁。根据 WHO 组织病理学分级，27 例患者为 2 级（54%），23 例患者为 3 级（46%）。30 例患者（60%）达到了大体全切除（GTR）。中位随访时间为 65 个月。5 年 PFS 和 OS 分别为 47.2±7.3%和 73.3±6.7%。GTR 与更长的 OS 相关（P=0.02）。组织病理学分级不是 OS 的独立预后因素。3 级患者的有丝分裂和微血管增生高于 2 级患者，这有助于确定 WHO 分级。这项全国性研究揭示了儿童室管膜瘤患者的特征和结局。GTR 是与生存改善最相关的因素。相比之下，该队列的组织病理学分级不是一个显著的预后因素。在未来的研究中，应该进一步寻求更具可重复性和实用性的颅内室管膜瘤诊断标准。
