Evans D G R, Bulman Mike, Young Karen, Howard Emma, Bayliss Stuart, Wallace Andrew, Lalloo Fiona
Academic Unit of Medical Genetics, National Genetics Reference Laboratory and Regional Genetic Service, St. Mary's Hospital, Hathersage Road, Manchester M13 0JH, UK.
Fam Cancer. 2008;7(2):113-7. doi: 10.1007/s10689-007-9153-9. Epub 2007 Jul 17.
64 families with a history of male breast cancer aged 60 or less or with a family history of male and female breast cancer were screened for the presence of BRCA1 and BRCA2 mutations. Seventeen pathogenic BRCA2 and four BRCA1 mutations were identified (34%) in samples from an affected family member. All but one of the mutations segregated with disease where samples were available and pedigree structure permitted. Despite high sensitivity of mutation testing only 64% of families fulfilling BCLC criteria had an identifiable pathogenic mutation. It is possible that at least some of these families may have mutations in other genes, although we found no involvement of CHEK2 1100delC.
对60岁及以下有男性乳腺癌家族史或有男性和女性乳腺癌家族史的64个家庭进行了BRCA1和BRCA2突变筛查。在一名受影响家庭成员的样本中鉴定出17个致病性BRCA2突变和4个BRCA1突变(34%)。在有样本且系谱结构允许的情况下,除一个突变外,所有突变均与疾病共分离。尽管突变检测具有高敏感性,但符合BCLC标准的家庭中只有64%有可识别的致病性突变。这些家庭中至少有一些可能在其他基因中有突变,尽管我们未发现CHEK2 1100delC基因受累。