Department of Computational Discovery, Foundation Medicine, Inc., Boston, MA, United States.
Department of Pathology, Foundation Medicine, Inc., Boston, MA, United States, and.
Oncologist. 2024 Aug 5;29(8):658-666. doi: 10.1093/oncolo/oyae149.
Despite recent efforts to issue clinical guidelines outlining strategies to define the pathogenicity of genomic variants, there is currently no standardized framework for which to make these assertions. This review does not present a step-by-step methodology, but rather takes a holistic approach to discuss many aspects which should be taken into consideration when determining variant pathogenicity. Categorization should be curated to reflect relevant findings within the scope of the specific medical context. Functional characterization should evaluate all available information, including results from literature reviews, different classes of genomic data repositories, and applicable computational predictive algorithms. This article further proposes a multidimensional view to infer pathogenic status from many genomic measurements across multiple axes. Notably, tumor suppressors and oncogenes exhibit fundamentally different biology which helps refine the importance of effects on splicing, mutation interactions, copy number thresholds, rearrangement annotations, germline status, and genome-wide signatures. Understanding these relevant datapoints with thoughtful perspective could aid in the reclassification of variants of unknown significance (VUS), which are ambiguously understood and currently have uncertain clinical implications. Ongoing assessments of VUS examining these relevant biological axes could lead to more accurate classification of variant pathogenicity interpretation in diagnostic oncology.
尽管最近已经努力制定了临床指南,概述了定义基因组变异致病性的策略,但目前还没有标准化的框架来做出这些断言。本文并非介绍一种逐步的方法,而是采用整体方法来讨论在确定变异致病性时应考虑的许多方面。分类应精心策划,以反映特定医学背景范围内的相关发现。功能表征应评估所有可用信息,包括文献综述、不同类别的基因组数据库和适用的计算预测算法的结果。本文进一步提出了一种多维视角,从多个轴上的多个基因组测量推断致病性状态。值得注意的是,肿瘤抑制基因和癌基因表现出根本不同的生物学特性,这有助于细化对剪接、突变相互作用、拷贝数阈值、重排注释、种系状态和全基因组特征的影响的重要性。从深思熟虑的角度理解这些相关数据点可以帮助重新分类意义不明的变异(VUS),这些变异的理解不明确,目前具有不确定的临床意义。对这些相关生物学轴进行的 VUS 持续评估可能导致在诊断肿瘤学中更准确地分类变异致病性解释。