Heald Brandie, Marquard Jessica, Funchain Pauline
Center for Personalized Genetic Healthcare, Genomic Medicine Institute, Cleveland Clinic, Cleveland, OH.
Center for Personalized Genetic Healthcare, Genomic Medicine Institute, Cleveland Clinic, Cleveland, OH.
Semin Oncol. 2016 Oct;43(5):609-614. doi: 10.1053/j.seminoncol.2016.08.008. Epub 2016 Sep 21.
Hereditary cancer syndromes generally account for 5%-10% of malignancies. While these syndromes are rare, affected patients carry significantly elevated risks of developing cancer, as do their at-risk relatives. Identification of these patients is critical to ensure timely and appropriate genetic testing relevant to cancer patients and their relatives. Several guidelines and tools are available to assist clinicians. Patients suspected to have hereditary cancer syndromes should be offered genetic testing in the setting of genetic counseling by a qualified genetics professional. Germline testing ranges from testing for a known specific familial mutation to testing of a broad differential diagnosis using a pan-cancer multi-gene panel. Taking a family history, referring specific types of tumors with higher likelihood of heredity, implementing universal screening protocols such as microsatellite instability/immunohistochemistry (MSI/IHC) for specific tumors, and referring patients with somatic tumor testing that have potentially germline consequences are all important components to the identification of hereditary cancer syndromes in the oncology clinic.
遗传性癌症综合征通常占所有恶性肿瘤的5%-10%。虽然这些综合征较为罕见,但患病患者及其高危亲属患癌风险会显著升高。识别这些患者对于确保为癌症患者及其亲属及时提供恰当的相关基因检测至关重要。有多项指南和工具可协助临床医生。疑似患有遗传性癌症综合征的患者应由合格的遗传学专业人员在遗传咨询过程中提供基因检测。种系检测范围从检测已知的特定家族性突变到使用泛癌多基因检测板进行广泛的鉴别诊断。采集家族病史、转诊具有较高遗传可能性的特定类型肿瘤、针对特定肿瘤实施通用筛查方案(如微卫星不稳定性/免疫组织化学(MSI/IHC))以及转诊进行过具有潜在种系后果的体细胞肿瘤检测的患者,都是肿瘤门诊识别遗传性癌症综合征的重要组成部分。