Laboratory of Molecular Radiology, Center for Disease Biology and Integrative Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
J Radiat Res. 2021 May 5;62(Supplement_1):i44-i52. doi: 10.1093/jrr/rrab009.
Large-scale cancer-associated gene testing is now being rapidly incorporated into clinical settings, and is leading to incidental identification of the germline variants present in cancer patients. Because many cancer susceptibility genes are related to DNA damage response and repair, the variants may reflect not only the susceptibility to cancer but also the genetically defined radiation sensitivity of the patients and their relatives. When the presence of a certain germline variant increases the risk for developing radiation toxicity or radiation-induced secondary cancers, it will greatly influence the clinical decision-making. In order to achieve optimal radiological risk communication and to select the best cancer management for a given patient based on information from gene testing, healthcare professionals including genetic counselors, risk communicators and clinicians need to increase their knowledge of the health effects of various genetic variants. While germline loss-of-function mutations in both of the alleles of the DNA damage response genes cause rare hereditary diseases characterized by extreme hypersensitivity to radiation, the health effects of the carriers who have germline variants in one allele of such genes would be a matter of debate, especially when the significance of the variants is currently unknown. In this review, we describe the clinical significance of the genetic variants of the important DNA damage response genes, including ATM and TP53, and discuss how we can apply current knowledge to the management of cancer patients and their relatives from a radiological point of view.
大规模癌症相关基因检测现已迅速纳入临床环境,导致偶然发现癌症患者中存在的种系变异。由于许多癌症易感性基因与 DNA 损伤反应和修复有关,这些变异不仅反映了癌症的易感性,还反映了患者及其亲属的遗传定义的辐射敏感性。当特定种系变异的存在增加了发生放射性毒性或放射性诱导的继发性癌症的风险时,它将极大地影响临床决策。为了实现最佳放射风险沟通,并根据基因检测信息为特定患者选择最佳癌症管理,包括遗传咨询师、风险沟通者和临床医生在内的医疗保健专业人员需要增加对各种遗传变异的健康影响的了解。虽然 DNA 损伤反应基因的两个等位基因中的种系功能丧失突变会导致罕见的遗传性疾病,其特征是对辐射极度敏感,但这些基因的一个等位基因中存在种系变异的携带者的健康影响将是一个有争议的问题,特别是当这些变异的意义目前尚不清楚时。在这篇综述中,我们描述了重要的 DNA 损伤反应基因(包括 ATM 和 TP53)的遗传变异的临床意义,并讨论了我们如何从放射学角度将当前的知识应用于癌症患者及其亲属的管理。