Ren Leihao, Deng Jiaojiao, Wakimoto Hiroaki, Xie Qing, Gong Ye, Hua Lingyang
Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Institute of Neurosurgery, Fudan University, Shanghai, China.
Acta Neuropathol Commun. 2025 Jan 24;13(1):15. doi: 10.1186/s40478-025-01925-0.
Meningioma represents the most common intracranial tumor in adults. However, it is rare in pediatric patients. We aimed to demonstrate the clinicopathological characteristics and long-term outcome of pediatric meningiomas (PMs).
We enrolled 74 patients with intracranial PMs and analyzed their clinicopathological characteristics. Targeted next generation sequencing was used to detect alterations in meningioma relevant genes. Progression-free survival (PFS) was compared between PMs and adult meningiomas (AMs). Univariate and multivariate Cox analyses were employed to evaluate the predictive values of clinicopathological characteristics. A nomogram was constructed and its predictive accuracy evaluated.
40 females (54.1%) and 34 males (45.9%) patients, with the gender ratio of 1.18:1, were identified. 9 (12.2%) cases were clinically diagnosed as NF2-related Schwannomatosis (NF2-SWN), while 65 (87.8%) were sporadic. Ventricular location was found in 16 patients (21.6%). 19 patients (25.7%) experienced recurrence during a median follow-up period of 33 months (range 2 -145.25 months). The 3-, 5-, and 8-year PFS rates was 74.74%, 74.74%, and 59.38%, respectively. The PFS of the PM and AM cohorts were not significantly different, with or without propensity score matching. NF2 mutation was observed in 33 sporadic PMs (52.4%), whereas alterations in other genes (AKT1, TRAF7, SMO, PIK3CA, KLF4) frequently mutated in AMs, were not identified. The proportion of NF2 mutation in PMs was significantly lower in the skull base than other locations (p = 0.02). One anaplastic PM harbored TERT promoter mutation. Of note, in sporadic PMs, NF2 mutations were not significantly associated with PFS (p = 0.434) or overall survival (OS) (p = 0.60). The multivariate Cox analysis showed NF2-SWN (p < 0.001) and extent of resection (p = 0.013) to be independently associated with the PFS of PMs. Our prognostic model showed predictive accuracy for long-term PFS in PMs as the 3-, 5- and 8-year Area Under the Curve (AUC) was 0.927, 0.930, and 0.870, respectively.
PM was characterized by its relative male predominance, ventricular location, NF2-SWN, and NF2 mutation. Of note, PMs had similar prognosis to AMs and NF2 alteration was not significantly associated with PFS in PMs.
脑膜瘤是成人最常见的颅内肿瘤。然而，在儿科患者中却很罕见。我们旨在阐述儿童脑膜瘤（PMs）的临床病理特征和长期预后。
我们纳入了74例颅内PMs患者，并分析了他们的临床病理特征。采用靶向二代测序检测脑膜瘤相关基因的改变。比较了PMs与成人脑膜瘤（AMs）的无进展生存期（PFS）。采用单因素和多因素Cox分析评估临床病理特征的预测价值。构建了列线图并评估其预测准确性。
共纳入40例女性（54.1%）和34例男性（45.9%）患者，性别比为1.18:1。9例（12.2%）临床诊断为与NF2相关的施万细胞瘤病（NF2-SWN），而65例（87.8%）为散发性。16例患者（21.6%）肿瘤位于脑室。19例患者（25.7%）在中位随访期33个月（范围2 - 145.25个月）内出现复发。3年、5年和8年的PFS率分别为74.74%、74.74%和59.38%。无论是否进行倾向得分匹配，PMs和AMs队列的PFS均无显著差异。在33例散发性PMs中观察到NF2突变（52.4%），而未发现AMs中常见的其他基因（AKT1、TRAF7、SMO、PIK3CA、KLF4）的改变。PMs中NF2突变在颅底的比例显著低于其他部位（p = 0.02）。1例间变性PMs存在TERT启动子突变。值得注意的是，在散发性PMs中，NF2突变与PFS（p = 0.434）或总生存期（OS）（p = 0.60）无显著相关性。多因素Cox分析显示NF2-SWN（p < 0.001）和切除范围（p = 0.013）与PMs的PFS独立相关。我们的预后模型对PMs的长期PFS显示出预测准确性，3年、5年和8年的曲线下面积（AUC）分别为0.927、0.930和0.870。
PMs的特征为相对男性居多、位于脑室、NF2-SWN和NF2突变。值得注意的是，PMs与AMs预后相似，且NF2改变与PMs的PFS无显著相关性。
