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小儿和成人恶性脑胶质瘤:近亲还是远房表亲?

Paediatric and adult malignant glioma: close relatives or distant cousins?

机构信息

Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, 15 Cotswold Road, Sutton SM2 5NG, UK.

出版信息

Nat Rev Clin Oncol. 2012 May 29;9(7):400-13. doi: 10.1038/nrclinonc.2012.87.

DOI:10.1038/nrclinonc.2012.87
PMID:22641364
Abstract

Gliomas in children differ from their adult counterparts by their distribution of histological grade, site of presentation and rate of malignant transformation. Although rare in the paediatric population, patients with high-grade gliomas have, for the most part, a comparably dismal clinical outcome to older patients with morphologically similar lesions. Molecular profiling data have begun to reveal the major genetic alterations underpinning these malignant tumours in children. Indeed, the accumulation of large datasets on adult high-grade glioma has revealed key biological differences between the adult and paediatric disease. Furthermore, subclassifications within the childhood age group can be made depending on age at diagnosis and tumour site. However, challenges remain on how to reconcile clinical data from adult patients to tailor novel treatment strategies specifically for paediatric patients.

摘要

儿童脑胶质瘤在组织学分级、发病部位和恶性转化速率方面与成人脑胶质瘤不同。虽然在儿童中较为罕见,但高级别脑胶质瘤患者的临床结局与形态学相似的老年患者相比,大多同样不容乐观。分子谱分析数据开始揭示这些儿童恶性肿瘤的主要遗传改变。事实上,大量关于成人高级别脑胶质瘤的数据集揭示了成人和儿童疾病之间的关键生物学差异。此外,还可以根据诊断时的年龄和肿瘤部位对儿童年龄组进行分类。然而,如何将成人患者的临床数据协调一致,以制定专门针对儿科患者的新治疗策略,仍然存在挑战。

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Mesenchymal transition and PDGFRA amplification/mutation are key distinct oncogenic events in pediatric diffuse intrinsic pontine gliomas.间质转化和 PDGFRA 扩增/突变是儿童弥漫性内在脑桥神经胶质瘤的关键独特致癌事件。
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Intratumoral heterogeneity of receptor tyrosine kinases EGFR and PDGFRA amplification in glioblastoma defines subpopulations with distinct growth factor response.
小儿脑癌治疗转化流程中的机遇。
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