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应对ALT阳性神经母细胞瘤:是时候重新定义风险分类系统了吗? 一个系统的审查与IPD元分析

Marta Avinent-Pérez¹, Frank Westermann², Samuel Navarro³

¹Department of Pathology, Medical School, University of Valencia, 46010 Valencia, Spain; Neuroblastoma Genomics, German Cancer Research Center (DKFZ), Heidelberg, Germany.

Neoplasia (New York, N.Y.)

|December 29, 2024

概括

带有替代端粒延长 (ALT) 的神经母细胞瘤患者的生存率较差. 将端粒维持机制 (TMM) 纳入风险分类可以改善高风险神经母细胞瘤的治疗.

科学领域:

在瘤学瘤学.
遗传学遗传学是一个
分子生物学分子生物学

背景情况:

神经母细胞瘤的预后有很大的变化,受端粒维护机制 (TMM) 的影响.
端粒的替代延长 (ALT) 途径是影响患者结果的关键TMM.
现有的风险分类系统对于高风险神经母细胞瘤患者缺乏准确性,特别是那些患有ALT的患者.

研究的目的:

对ALT阳性神经母细胞瘤患者的生存进行个人参与者数据 (IPD) 分析.
通过纳入TMM状态来完善神经母细胞瘤的风险分类系统.
增强治疗策略和改善患者的治疗结果.

主要方法:

按照PRISMA-IPD指导方针进行了一次系统的文献审查.
数据从PubMed,Scopus和Embase数据库中提取到2024年3月的数据.
患者被分为ALT阳性和TMM阴性分组,以评估整体和无事件生存期.

主要成果:

分析包括来自8项研究的293名神经母细胞瘤患者 (156名ALT阳性,137名TMM阴性).
与TMM阴性患者相比,ALT阳性患者的整体和无事件生存率明显较低.
在非4期和4期ALT阳性患者中观察到较低的生存率,突出显示了ALT的预后意义.

结论:

使用ALT途径的神经母细胞瘤患者的结果明显较差.
将TMM状态,特别是ALT,纳入国际风险分类是准确预测的关键.
标准化TMM评估方法对于制定针对ALT阳性神经母细胞瘤的量身定制治疗策略至关重要.

关键词:

儿童癌症儿童癌症预后生物标志物预后生物标志物幸存的生存方式端粒维护机制端粒维护机制治疗策略治疗策略

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