Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma.

Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma.
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DOI:
10.1016/j.ccr.2011.07.007
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发表时间:
2011-08-16
期刊:
影响因子:
50.3
通讯作者:
Pfister SM
Pfister SM
中科院分区:
医学1区
文献类型:
--
作者:
Witt H;Mack SC;Ryzhova M;Bender S;Sill M;Isserlin R;Benner A;Hielscher T;Milde T;Remke M;Jones DT;Northcott PA;Garzia L;Bertrand KC;Wittmann A;Yao Y;Roberts SS;Massimi L;Van Meter T;Weiss WA;Gupta N;Grajkowska W;Lach B;Cho YJ;von Deimling A;Kulozik AE;Witt O;Bader GD;Hawkins CE;Tabori U;Guha A;Rutka JT;Lichter P;Korshunov A;Taylor MD;Pfister SM

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尽管室管膜瘤在整个神经轴的组织学上相似,但这种疾病可能包括多个独立的实体,每个实体都有不同的分子发病机制。两个大型独立室管膜瘤队列的转录谱显示存在两组在人口统计学、转录、遗传和临床上不同的后颅窝(PF)室管膜瘤。A组患者更年轻,肿瘤位于侧面,基因组平衡,与B组患者相比,更容易出现复发、复发时转移和死亡。鉴定和优化PF室管膜瘤亚组的免疫组化(IHC)标记物,可以验证我们在第三个独立队列中的发现,使用人类室管膜瘤组织微阵列,并提供了一个工具,用于PF室管膜瘤患者的前瞻性分类和分层。
Despite the histological similarity of ependymomas from throughout the neuroaxis, the disease likely comprises multiple independent entities, each with a distinct molecular pathogenesis. Transcriptional profiling of two large independent cohorts of ependymoma reveals the existence of two demographically, transcriptionally, genetically, and clinically distinct groups of posterior fossa (PF) ependymomas. Group A patients are younger, have laterally located tumors with a balanced genome, and are much more likely to exhibit recurrence, metastasis at recurrence, and death compared with Group B patients. Identification and optimization of immunohistochemical (IHC) markers for PF ependymoma subgroups allowed validation of our findings on a third independent cohort, using a human ependymoma tissue microarray, and provides a tool for prospective prognostication and stratification of PF ependymoma patients.
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