Zhang Min, Chen Tianhui
School of Public Health, Hangzhou Medical College, Hangzhou, Zhejiang, China.
Department of Cancer Prevention/Experimental Research Center, Cancer Hospital of the University of Chinese Academy of Sciences (Zhejiang Cancer Hospital), Institute of Basic Medicine and Cancer (IBMC), Chinese Academy of Sciences, Hangzhou, China.
Hered Cancer Clin Pract. 2021 May 1;19(1):26. doi: 10.1186/s13053-021-00182-1.
DNA mismatch repair (MMR) genes play an important role in maintaining genome stability. Germline mutations in MMR genes disrupt the mismatch repair function and cause genome instability. Carriers with MMR germline mutations are more likely to have MMR deficiency and microsatellite instability (MSI) than non-carriers and are prone to develop colorectal cancer (CRC) and extracolorectal malignancies, known as Lynch syndrome (LS). MMR gene testing for suspected mutation carriers is a reliable method to identify the mutation types and to discover mutation carriers. Given that carriers of MMR germline mutations have a higher risk of LS-related cancers (LS-RC) and a younger age at onset than non-carriers, early surveillance and regular screening of relevant organs of carriers are very important for early detection of related cancers. This review mainly focuses on the general status of MMR carriers, the approaches for early detection and screening, and the surveillance of MMR mutation carriers in China. Population screening of MMR germline mutation carriers in China will be helpful for early detection, early diagnosis and treatment of MMR mutation carriers, which may improve the 5-year survival, and reduce mortality and incidence rate in the long term.
DNA错配修复(MMR)基因在维持基因组稳定性方面发挥着重要作用。MMR基因的种系突变会破坏错配修复功能,导致基因组不稳定。与非携带者相比,携带MMR种系突变的个体更易出现MMR缺陷和微卫星不稳定(MSI),并且容易发生结直肠癌(CRC)和结直肠外恶性肿瘤,即林奇综合征(LS)。对疑似突变携带者进行MMR基因检测是识别突变类型和发现突变携带者的可靠方法。鉴于MMR种系突变携带者患LS相关癌症(LS-RC)的风险更高且发病年龄比非携带者更小,对携带者的相关器官进行早期监测和定期筛查对于早期发现相关癌症非常重要。本综述主要聚焦于中国MMR携带者的总体状况、早期检测和筛查方法以及MMR突变携带者的监测。在中国对MMR种系突变携带者进行人群筛查将有助于对MMR突变携带者进行早期发现、早期诊断和治疗,这可能提高5年生存率,并从长期来看降低死亡率和发病率。