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Glioblastoma with oligodendroglioma component (GBM-O): Molecular genetic and clinical characteristics

机译:胶质母细胞瘤与少突胶质细胞瘤成分(GBM-O):分子遗传学和临床特征

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Glioblastoma (GBM) is an aggressive primary brain tumor with an average survival of approximately 1 year. A recently recognized subtype, glioblastoma with oligodendroglioma component (GBM-O), was designated by the World Health Organization (WHO) in 2007. We investigated GBM-Os for their clinical and molecular characteristics as compared to other forms of GBM. Tissue samples were used to determine EGFR, PTEN, and 1p and 19q status by fluorescence in situ hybridization (FISH); p53 and mutant IDH1 protein expression by immunohistochemistry (IHC); and MGMT promoter status by methylation-specific polymerase chain reaction (PCR). GBM-Os accounted for 11.9% of all GBMs. GBM-Os arose in younger patients compared to other forms of GBMs (50.7 years vs. 58.7 years, respectively), were more frequently secondary neoplasms, had a higher frequency of IDH1 mutations and had a lower frequency of PTEN deletions. Survival was longer in patients with GBM-Os compared to those with other GBMs, with median survivals of 16.2 and 8.1 months, respectively. Most of the survival advantage for GBM-O appeared to be associated with a younger age at presentation. Among patients with GBM-O, younger age at presentation and 1p deletion were most significant in conferring prolonged survival. Thus, GBM-O represents a subset of GBMs with distinctive morphologic, clinical and molecular characteristics.
机译:胶质母细胞瘤(GBM)是一种侵袭性原发性脑肿瘤,平均生存期约为1年。 2007年,世界卫生组织(WHO)指定了一种新近公认的亚型,具有少突胶质细胞瘤成分的胶质母细胞瘤(GBM-O)。与其他形式的GBM相比,我们调查了GBM-Os的临床和分子特征。使用组织样本通过荧光原位杂交(FISH)测定EGFR,PTEN以及1p和19q状态;通过免疫组织化学(IHC)检测p53和IDH1突变蛋白的表达;甲基化特异性聚合酶链反应(PCR)检测MGMT启动子的状态。 GBM-O占所有GBM的11.9%。与其他形式的GBM相比,年轻患者中出现的GBM-Os(分别为50.7岁和58.7岁),是继发性肿瘤,IDH1突变频率较高,PTEN缺失频率较低。与其他GBM相比,GBM-O患者的生存期更长,中位生存期分别为16.2和8.1个月。 GBM-O的大多数生存优势似乎与出现时年龄较小有关。在患有GBM-O的患者中,出现年龄提早和1p缺失对延长生存期最重要。因此,GBM-0代表具有独特的形态,临床和分子特征的GBM的子集。

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