Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing , China.
Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing , China.
Neurosurgery. 2024 Sep 1;95(3):651-659. doi: 10.1227/neu.0000000000002923. Epub 2024 Mar 26.
Posterior fossa ependymomas (PFEs) are rare brain tumors classified as PF-EPN-A (PFA) and PF-EPN-B (PFB) subgroups. The study aimed to evaluate the prognosis and survival outcomes in PFEs, with a focus on the impact of molecular subgroups.
A retrospective study was conducted on 412 patients with PFEs. Kaplan-Meier survival analyses were conducted to evaluate the overall survival (OS) and progression-free survival. Cox regression analyses were conducted to assess the prognostic factors. A nomogram was developed to predict the OS rates of PFEs.
The study revealed significant differences between PFA and PFB in patient and tumor characteristics. PFAs were associated with poorer OS (hazard ratios [HR] 3.252, 95% CI 1.777-5.950, P < .001) and progression-free survival (HR 4.144, 95% CI 2.869-5.985, P < .001). World Health Organization grade 3 was associated with poorer OS (HR 2.389, 95% CI 1.236-4.617, P = .010). As for treatment patterns, gross total resection followed by radiotherapy or the combination of radiotherapy and chemotherapy yielded the most favorable OS for PFA ( P = .025 for both), whereas gross total resection followed by radiotherapy rather than observation showed improved OS for PFB ( P = .046). The nomogram demonstrated a high degree of accuracy and discrimination capacity for the prediction of OS rates for up to 10 years. In addition, 6 cases of PFA (3.51%) with H3K27M mutations were identified.
PFAs demonstrate worse prognosis and survival outcomes compared with PFBs. Both PFAs and PFBs necessitate maximal resection followed by intensive adjuvant therapies in long-term effects.
后颅窝室管膜瘤（PFEs）是一种罕见的脑肿瘤，分为 PF-EPN-A（PFA）和 PF-EPN-B（PFB）亚组。本研究旨在评估 PFEs 的预后和生存结果，并重点关注分子亚组的影响。
对 412 例 PFEs 患者进行回顾性研究。采用 Kaplan-Meier 生存分析评估总生存期（OS）和无进展生存期。采用 Cox 回归分析评估预后因素。建立列线图预测 PFEs 的 OS 率。
研究显示 PFA 和 PFB 在患者和肿瘤特征方面存在显著差异。PFA 与较差的 OS（风险比 [HR] 3.252，95%CI 1.777-5.950，P <.001）和无进展生存期（HR 4.144，95%CI 2.869-5.985，P <.001）相关。世界卫生组织（WHO）分级 3 级与较差的 OS 相关（HR 2.389，95%CI 1.236-4.617，P =.010）。至于治疗模式，肉眼全切除后行放疗或放疗联合化疗对 PFA 最有利（P =.025，两者均有），而肉眼全切除后行放疗而非观察对 PFB 有更好的 OS（P =.046）。列线图对 OS 率的预测具有较高的准确性和区分能力，可预测长达 10 年的结果。此外，还发现了 6 例 PFA（3.51%）存在 H3K27M 突变。
与 PFB 相比，PFA 预后和生存结果更差。PFAs 和 PFBs 均需要最大限度地切除，然后进行长期强化辅助治疗。
