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Primary cerebellar glioblastomas in children: clinical presentation and management.
Neurosurgical Review ( IF 2.8 ) Pub Date : 2020-08-26 , DOI: 10.1007/s10143-020-01373-5
Qiguang Wang 1 , Jian Cheng 1 , Zhang Si 1 , Wenke Liu 1 , Xuhui Hui 1 , Qiang Li 1 , Yan Ju 1
Affiliation  

Pediatric cerebellar glioblastomas (pcGBMs) are rare and their characteristics remain ill-defined. We conducted a retrospective analysis of pediatric cerebellar glioblastomas who underwent surgery from 2008 to 2019 in our department. Besides, we performed a literature review of the literature data on pcGBMs. Ten children with mean age of 9.4 years were included. During the follow-up, six patients died with mean survival time of 11.7 months, four patients survived with mean follow-up of 28 months. Seven patients underwent molecular analysis, no patients detected IDH1 mutations, four patients (57.1%) had H3K27M mutations, and two patients (28.6%) had MGMT promoter methylation. The literature review identified 38 pcGBMs cases (including ours), with mean age of 8.84 ± 4.20 years (range, 1–16 years). Increased ICP was the commonest sign. Eighteen (47.4%) patients underwent GTR and fifteen (45.5%) patients received STR. Postoperative radiation (RT) was conducted in 28 patients (75.7%) and 23 patients (65.7%) received chemotherapy. During the follow-up, 25 patients died with mean survival time of 12.21 months and 11 patients survived with average follow-up of 29.3 months. Kaplan-Meier survival depicted chemotherapy (P < 0.001) or radiation (P < 0.001) had positive impact on overall survival. Multivariate analysis revealed chemotherapy was a significant predictor of survival with a hazard ratio of 3.264 (P = 0.038). Our study found mean overall survival time for pcGBMs patients was 12.21 months. PcGBMs may have distinct molecular features, with higher incidence of H3K27M mutation and were always IDH1 wild-type. We recommend the routine postoperative radiotherapy and chemotherapy in pcGBMs.



中文翻译:

儿童原发性小脑胶质母细胞瘤:临床表现和治疗。

小儿小脑胶质母细胞瘤(pcGBM)很少见,其特征仍不清楚。我们对我科2008年至2019年接受手术的小儿小脑胶质母细胞瘤进行了回顾性分析。此外,我们对pcGBMs的文献数据进行了文献综述。包括十名平均年龄为9.4岁的儿童。在随访期间,有6例患者死亡,平均生存时间为11.7个月,有4例患者存活,平均随访时间为28个月。7名患者接受了分子分析,没有患者检测到IDH1突变,4名患者(57.1%)有H3K27M突变,2名患者(28.6%)有MGMT启动子甲基化。文献综述确定了38例pcGBMs病例(包括我们的病例),平均年龄为8.84±4.20岁(范围1–16岁)。ICP增加是最常见的迹象。十八(47。4%的患者接受了GTR,十五(45.5%)例接受了STR。术后放疗(RT)的28例(75.7%)和23例(65.7%)接受了化疗。在随访期间,有25例患者死亡,平均生存时间为12.21个月,有11例患者存活,平均随访时间为29.3个月。Kaplan-Meier生存率描述了化疗(P  <0.001)或放射线(P  <0.001)对总体生存有积极影响。多变量分析显示,化疗是生存率的重要预测指标,危险比为3.264(P  = 0.038)。我们的研究发现pcGBMs患者的平均总生存时间为12.21个月。PcGBMs可能具有独特的分子特征,H3K27M突变的发生率更高,并且始终是IDH1野生型。我们建议在pcGBM中进行常规术后放疗和化疗。

更新日期:2020-08-26
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