髓母细胞瘤亚群的分子和临床相关性:一篇叙述性综述

Glioma Pub Date : 2021-10-01 DOI:10.4103/glioma.glioma_18_21
Hallie Coltin, V. Ramaswamy
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引用次数: 1

摘要

髓母细胞瘤是儿童癌症相关发病率和死亡率的主要原因,因为相当大比例的患者死于疾病,大多数幸存者都会留下终身的治疗后遗症。以前的髓母细胞瘤分类系统严重依赖组织学,未能考虑肿瘤生物学。即将于2021年世界卫生组织对中枢神经系统肿瘤进行的分类现在坚定地表明,髓母细胞瘤实际上包括至少四个不同的分子实体,每组中都有相当多的亚结构。当代临床试验的研究设计首次认识到髓母细胞瘤的分子异质性。将常规分子亚组纳入即将进行的临床试验有可能显著提高被诊断为髓母细胞瘤的儿童和成人的生存率和生活质量。本综述旨在总结髓母细胞瘤基因组学的最新进展,并总结已发表的分子知情临床试验的及时结果。具体而言,除了SJMB03、ACNS0331和ACNS0332的结果外,还将审查英语文献。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Molecular and clinical correlates of medulloblastoma subgroups: A narrative review
Medulloblastoma is a major cause of cancer-related morbidity and mortality in children, as a significant proportion of patients succumb to their disease and most survivors are left with life-long sequelae of therapy. Prior medulloblastoma classification systems relied heavily on histology and failed to account for tumor biology. The upcoming 2021 WHO classification of central nervous system tumors now firmly establishes that medulloblastoma actually comprises at least four distinct molecular entities, with considerable substructure within each group. For the first time, the study design of contemporary clinical trials has now recognized the molecular heterogeneity of medulloblastoma. The incorporation of routine molecular subgrouping into upcoming clinical trials has the potential to significantly improve survival and quality of life for children and adults diagnosed with medulloblastoma. This review was conducted to summarize these recent advances in the genomics of medulloblastoma and to summarize the timely results of molecularly-informed published clinical trials. Specifically, English language literature will be reviewed in addition to the results of SJMB03, ACNS0331, and ACNS0332.
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