Department of Neurosurgery, All India Institute of Medical Sciences, Raebareli, Uttar Pradesh, India, 229 405.
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India, 500 082.
Childs Nerv Syst. 2024 Jan;40(1):65-71. doi: 10.1007/s00381-023-06095-9. Epub 2023 Aug 30.
Diffuse Midline Glioma (DMG) with H3K27M mutation is a rare and aggressive midline high grade glioma with a predominant astrocytic differentiation and K27M mutation in either H3F3A or HIST1H3B/C. This tumor is more common in children than in adults. The current study was aimed to determine clinicohistoradiological and surgical outcome of patients who have undergone surgery for DMG and study disease severity of patients with DMG.
This is an observational study in which 29 DMG patients were evaluated for clinicohistoradiological and surgical outcomes by assessing the pre and postoperative neurological status.
Survival duration was significantly high in patients with age > 18 years (p = 0.02). Patients who had undergone Radiation Therapy showed higher survival rate (p = 0.05) and the cases with low levels of Ki 67 index had improved post operative outcome (p = 0.002).
DMG with H3K27M mutation in newly classified Central Nervous System tumor are WHO grade IV Tumors, comprising H3K27M mutation as molecular marker for diagnosis and related with a poor prognosis.
弥漫性中线胶质瘤（DMG）伴 H3K27M 突变是一种罕见且侵袭性的中线高级别胶质瘤，主要为星形细胞分化，并在 H3F3A 或 HIST1H3B/C 中发生 K27M 突变。这种肿瘤在儿童中比在成人中更常见。本研究旨在确定接受 DMG 手术的患者的临床病理和手术结果，并研究 DMG 患者的疾病严重程度。
这是一项观察性研究，通过评估术前和术后的神经状态，评估 29 名 DMG 患者的临床病理和手术结果。
年龄>18 岁的患者生存时间明显较长（p=0.02）。接受放射治疗的患者生存率更高（p=0.05），Ki-67 指数较低的病例术后预后更好（p=0.002）。
新分类的中枢神经系统肿瘤中伴 H3K27M 突变的 DMG 是 WHO 分级 IV 肿瘤，包括 H3K27M 突变作为诊断的分子标志物，并与预后不良相关。
