Zhong Shuai, Zuo Jinyi, Fu Xiaojun, Wu Chenxing, Liu Rui, Huang Zheng, Li Shouwei
Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, 100093, China.
J Neurooncol. 2025 Apr;172(2):437-445. doi: 10.1007/s11060-024-04933-7. Epub 2025 Jan 15.
This study aimed to describe the incidence, clinical and pathological features, and outcomes of H3 K27M- mutant Diffuse Midline Glioma (DMG) patients with leptomeningeal dissemination (LMD) and systematically investigate the predictive and prognostic factors to clarify the response to treatment after the onset of LMD.
A total of 304 patients diagnosed with DMG from October 17, 2017, to October 17, 2023, were enrolled in this study, of which 32 patients were diagnosed with LMD. Logistic regression analyses were conducted to identify the predictors of LMD, including clinical, molecular, and imaging data. Univariable and multivariable cox regression analyses were used for overall survival (OS) and post-LMD survival (PLS) analysis.
The median OS and PLS were 12.5 and 8.0 months respectively. Tumor with contrast-enhanced lesions reaching ependyma (Ventricular contact type I) was the only independent risk factor for LMD. Male sex and ventricular contact type I were independent risk factors for primary LMD. In all LMD patients, Karnofsky Performance Status (KPS) of ≥ 90 and radiotherapy were statistically significantly associated with longer OS, and primary LMD was significantly associated with shorter OS. Supratentorial location and chemotherapy after LMD diagnosis were independent favorable prognostic factors on PLS. In primary LMD subgroup analysis, radiotherapy was the only independent favorable prognostic factor on OS.
The association between contrast-enhanced lesions and ventricular involvement is an independent predictive factor for LMD in DMG patients. Radiotherapy and preoperative KPS may contribute to improved overall survival in these patients. Chemotherapy is a potential treatment option following an LMD diagnosis.
本研究旨在描述伴有软脑膜播散（LMD）的H3 K27M突变型弥漫性中线胶质瘤（DMG）患者的发病率、临床和病理特征及预后，并系统研究预测和预后因素，以阐明LMD发生后的治疗反应。
本研究纳入了2017年10月17日至2023年10月17日期间诊断为DMG的304例患者，其中32例诊断为LMD。进行逻辑回归分析以确定LMD的预测因素，包括临床、分子和影像学数据。单变量和多变量cox回归分析用于总生存期（OS）和LMD后生存期（PLS）分析。
中位OS和PLS分别为12.5个月和8.0个月。伴有增强病变累及室管膜（脑室接触I型）的肿瘤是LMD的唯一独立危险因素。男性和脑室接触I型是原发性LMD的独立危险因素。在所有LMD患者中，卡氏功能状态（KPS）≥90和放疗与较长的OS在统计学上显著相关，原发性LMD与较短的OS显著相关。幕上位置和LMD诊断后化疗是PLS的独立有利预后因素。在原发性LMD亚组分析中，放疗是OS的唯一独立有利预后因素。
增强病变与脑室受累之间的关联是DMG患者LMD的独立预测因素。放疗和术前KPS可能有助于改善这些患者的总生存期。化疗是LMD诊断后的一种潜在治疗选择。
