Cybulski C, Lubiński J, Wokołorczyk D, Kuźniak W, Kashyap A, Sopik V, Huzarski T, Gronwald J, Byrski T, Szwiec M, Jakubowska A, Górski B, Dębniak T, Narod S A, Akbari M R
Department of Genetics and Pathology, International Hereditary Cancer Center, Pomeranian Medical University, Szczecin, Poland.
Women's College Research Institute, Women's College Hospital, University of Toronto, Toronto, Canada.
Clin Genet. 2015 Oct;88(4):366-70. doi: 10.1111/cge.12524. Epub 2014 Nov 13.
A number of genes other than BRCA1 and BRCA2 have been associated with breast cancer predisposition, and extended genetic testing panels have been proposed. It is of interest to establish the full spectrum of deleterious mutations in women with familial breast cancer.We performed whole-exome sequencing of 144 women with familial breast cancer and negative for 11 Polish founder mutations in BRCA1, CHEK2 and NBS1, and we evaluated the sequences of 12 known breast cancer susceptibility genes. A truncating mutation in a breast cancer gene was detected in 24 of 144 women (17%) with familial breast cancer. A BRCA2 mutation was detected in 12 cases, a (non-founder) BRCA1 mutation was detected in 5 cases, a PALB2 mutation was detected in 4 cases and an ATM mutation was detected in 2 cases. Polish women with familial breast cancer who are negative for founder mutations in BRCA1, CHEK2 and NBS1 should be fully screened for mutations in BRCA1, BRCA2 and PALB2. The PALB2 founder mutation c.509_519delGA should be included in the panel of Polish founder mutations.
除BRCA1和BRCA2之外,还有一些基因与乳腺癌易感性相关,并且有人提出了扩展的基因检测方案。确定家族性乳腺癌女性中有害突变的全谱很有意义。我们对144例家族性乳腺癌女性进行了全外显子组测序,这些女性对BRCA1、CHEK2和NBS1中的11种波兰常见突变呈阴性,并且我们评估了12种已知乳腺癌易感基因的序列。在144例家族性乳腺癌女性中有24例(17%)检测到乳腺癌基因中的截短突变。12例检测到BRCA2突变,5例检测到(非常见)BRCA1突变,4例检测到PALB2突变,2例检测到ATM突变。对于BRCA1、CHEK2和NBS1常见突变呈阴性的波兰家族性乳腺癌女性,应全面筛查BRCA1、BRCA2和PALB2中的突变。波兰常见突变组应包括PALB2常见突变c.509_519delGA。