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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Pediatric glioblastoma with oligodendroglioma component: aggressive clinical phenotype with distinct molecular characteristics.
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Pediatric glioblastoma with oligodendroglioma component: aggressive clinical phenotype with distinct molecular characteristics.

机译：具有少突胶质细胞瘤成分的小儿胶质母细胞瘤：具有明显分子特征的侵袭性临床表型。

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The 2007 World Health Organization classification defined a new variant of glioblastoma (GBM) containing oligodendroglioma foci as GBM with an oligodendroglioma component (GBMO), which shows a favorable clinical outcome compared with "classic" GBM. However, all of the reported cases of GBMO have been adult cases, with no previous reports of pediatric cases. In this report, we demonstrated molecular characteristics of a pediatric GBMO case, showing aggressive clinical behavior with 8-month overall survival. The case showed neither isocitrate dehydrogenase 1/2 genes (IDH1/2) mutation nor 1p/19q co-deletion, a hallmark of oligodendroglioal tumors. In addition, microsatellite instability, leading to the putative mechanism of temozolomide (TMZ) resistance, was frequently detected. Molecular genetic analysis may provide critical prognostic and therapeutic insights, especially for the pediatric glioma containing oligodendroglioma components.
机译：2007年世界卫生组织的分类定义了一种胶质母细胞瘤（GBM）的新变体，其中含有少突胶质细胞瘤病灶和少突胶质细胞瘤成分（GBMO），与“经典” GBM相比，显示出良好的临床疗效。但是，所有报告的GBMO病例均为成人病例，以前没有小儿病例的报道。在本报告中，我们展示了小儿GBMO病例的分子特征，显示了具有侵略性的临床行为，总生存期为8个月。该病例既未显示异柠檬酸脱氢酶1/2基因（IDH1 / 2）突变，也未显示1p / 19q共缺失，这是少突神经胶质瘤的标志。此外，经常检测到微卫星不稳定性，导致推定的替莫唑胺（TMZ）抗性机制。分子遗传学分析可能提供重要的预后和治疗见解，特别是对于包含少突胶质细胞瘤成分的小儿神经胶质瘤。

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