Shields Lisa B, Daniels Michael W, Coombs Lennea, Vaynerman Alexandra, Sinicrope Kaylyn, Barbour Mustafa, Spalding Aaron, Gump William, Mutchnick Ian, Sun David
Norton Neuroscience Institute, Norton Healthcare, Louisville, USA.
Department of Bioinformatics and Biostatistics, University of Louisville, Louisville, USA.
Cureus. 2025 Feb 20;17(2):e79362. doi: 10.7759/cureus.79362. eCollection 2025 Feb.
Background Diffuse midline gliomas (DMG) are a subset of malignant gliomas that could be linked to an H3K27M mutation. Hydrocephalus may be the initial presenting condition because of its frequent pontine location. This study evaluated the outcomes of tumor resection, endoscopic third ventriculostomy (ETV), and ventriculoperitoneal shunt (VPS) placement in DMG patients compared to wild-type (WT) tumors in treating hydrocephalus. Materials and methods We identified newly diagnosed pediatric and adult patients with midline tumors over an eight-year period (September 14, 2016-July 1, 2024). Results Out of a total of 74 patients, 20 (27.0%) patients were diagnosed with an H3K27M mutation, and 24 (32.4%) presented with hydrocephalus. Patients with a DMG H3K27M mutant (15 patients) were statistically more likely to be diagnosed with hydrocephalus compared to those with a WT midline glioma (9 patients) (p<0.001). Of the 24 patients with hydrocephalus, 8 (34.8%) underwent a VPS placement, 5 (22.7%) had tumor resection, 5 (21.7%) underwent an ETV, and 4 (17.4%) had both a VPS and ETV. A significant difference in hydrocephalus-free survival was observed among the treatment groups (p=0.0013). ETV failure was significantly higher in H3K27M patients while VPS was more successful in managing hydrocephalus. Conclusions As H3K27M mutation analysis is not available rapidly when patients initially present with midline gliomas, neurosurgeons use their best clinical judgment regarding the management of hydrocephalus. VPS demonstrated superior outcomes compared to ETV in controlling hydrocephalus among patients with a DMG H3K27M mutation in the present study; therefore, neurosurgical teams should have increased vigilance following ETV in this population.
弥漫性中线胶质瘤（DMG）是恶性胶质瘤的一个子集，可能与H3K27M突变有关。由于其常位于脑桥，脑积水可能是最初的表现症状。本研究评估了DMG患者与野生型（WT）肿瘤患者在治疗脑积水时肿瘤切除、内镜下第三脑室造瘘术（ETV）和脑室腹腔分流术（VPS）的治疗效果。材料和方法：我们确定了在八年期间（2016年9月14日至2024年7月1日）新诊断的患有中线肿瘤的儿科和成年患者。结果：在总共74例患者中，20例（27.0%）被诊断为H3K27M突变，24例（32.4%）出现脑积水。与WT中线胶质瘤患者（9例）相比，DMG H3K27M突变患者（15例）在统计学上更易被诊断为脑积水（p<0.001）。在24例脑积水患者中，8例（34.8%）接受了VPS植入，5例（22.7%）进行了肿瘤切除，5例（21.7%）接受了ETV，4例（17.4%）同时接受了VPS和ETV。各治疗组间无脑积水生存期存在显著差异（p=0.0013）。H3K27M患者的ETV失败率显著更高，而VPS在治疗脑积水方面更成功。结论：当患者最初表现为中线胶质瘤时，由于无法迅速进行H3K27M突变分析，神经外科医生在脑积水管理方面需运用最佳临床判断。在本研究中，VPS在控制DMG H3K27M突变患者的脑积水方面显示出优于ETV的效果；因此，神经外科团队应对该人群ETV术后提高警惕。
