Primary cerebellar glioblastomas in children: clinical presentation and management
Primary cerebellar glioblastomas in children: clinical presentation and management
复制标题
儿童原发性小脑胶质母细胞瘤：临床表现和治疗
DOI：
10.1007/s10143-020-01373-5
复制
发表时间：
2020-08
影响因子：
2.8
通讯作者：
Yan Ju
中科院分区：
文献类型：
--
作者：
Qiguang Wang;Jian Cheng;Zhang Si;Wenke Liu;Xuhui Hui;Qiang Li;Yan Ju
小儿小脑胶质母细胞瘤（pcGBM）是罕见的，他们的特点仍然不明确。我们对2008年至2019年在我科接受手术的儿童小脑胶质母细胞瘤进行了回顾性分析。此外，我们对pcGBM的文献数据进行了文献综述。纳入了10名平均年龄为9.4岁的儿童。随访期间，6例患者死亡，平均生存时间为11.7个月，4例患者存活，平均随访时间为28个月。7例患者进行了分子分析，没有患者检测到IDH 1突变，4例患者（57.1%）有H3 K27 M突变，2例患者（28.6%）有MGMT启动子甲基化。文献综述确定了38例pcGBM病例（包括我们的病例），平均年龄为8.84 ± 4.20岁（范围，1-16岁）。颅内压增高是最常见的体征。18例（47.4%）患者接受了GTR，15例（45.5%）患者接受了STR。28例（75.7%）患者接受了术后放疗（RT），23例（65.7%）患者接受了化疗。随访期间，25例患者死亡，平均生存时间为12.21个月，11例患者存活，平均随访时间为29.3个月。Kaplan-Meier生存率显示化疗（P< 0.001）或放疗（P< 0.001）对总生存率有积极影响。多因素分析显示化疗是生存率的显著预测因素，风险比为3.264（P= 0.038）。我们的研究发现pcGBM患者的平均总生存时间为12.21个月。PcGBM可能具有独特的分子特征，具有较高的H3 K27 M突变发生率，并且总是IDH 1野生型。我们建议pcGBM术后常规放疗和化疗。
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.
登录
查看更多内容
影响因子：
8.8
作者：
Karremann M;Rausche U;Roth D;Kühn A;Pietsch T;Gielen GH;Warmuth-Metz M;Kortmann RD;Straeter R;Gnekow A;Wolff JE;Kramm CM
通讯作者：
Kramm CM
影响因子：
2.4
作者：
Jun Shinoda;Hiromu Yamada;Noboru Sakai;T. Ando;T. Hirata;H. Hirayama
通讯作者：
Jun Shinoda;Hiromu Yamada;Noboru Sakai;T. Ando;T. Hirata;H. Hirayama
影响因子：
12.7
作者：
Nomura M;Mukasa A;Nagae G;Yamamoto S;Tatsuno K;Ueda H;Fukuda S;Umeda T;Suzuki T;Otani R;Kobayashi K;Maruyama T;Tanaka S;Takayanagi S;Nejo T;Takahashi S;Ichimura K;Nakamura T;Muragaki Y;Narita Y;Nagane M;Ueki K;Nishikawa R;Shibahara J;Aburatani H;Saito N
通讯作者：
Saito N
影响因子：
15.8
作者：
Moher D;Liberati A;Tetzlaff J;Altman DG;PRISMA Group
通讯作者：
PRISMA Group
影响因子：
1.1
作者：
Tauziede-Espariat, Arnault;Saffroy, Raphael;Varlet, Pascale
通讯作者：
Varlet, Pascale