Spugnesi Laura, Gabriele Michele, Scarpitta Rosa, Tancredi Mariella, Maresca Luisa, Gambino Gaetana, Collavoli Anita, Aretini Paolo, Bertolini Ilaria, Salvadori Barbara, Landucci Elisabetta, Fontana Andrea, Rossetti Elena, Roncella Manuela, Naccarato Giuseppe Antonio, Caligo Maria Adelaide
Section of Genetic Oncology, Department of Laboratory Medicine, University Hospital of Pisa, Pisa, Italy.
Pisa Science Foundation Onlus, Pisa, Italy.
Genes Chromosomes Cancer. 2016 Dec;55(12):915-924. doi: 10.1002/gcc.22389. Epub 2016 Jul 26.
Triple negative breast cancers (TNBCs) represent about 15-20% of all breast cancer cases and are characterized by a complex molecular heterogeneity. Some TNBCs exhibit clinical and pathological properties similar to BRCA-mutated tumors, without actually bearing a mutation in BRCA genes. This "BRCAness" phenotype may be explained by germline mutations in other genes involved in DNA repair. Although respond to chemotherapy with alkylating agents, they have a high risk of recurrence and progression. Some studies have shown the efficacy of neoadjuvant therapy in TNBC patients with DNA repair defects, but proper biomarkers of DNA repair deficiency are still needed. Here, we investigated if mutations in DNA repair genes may be correlated with anthracyclines/taxanes neoadjuvant therapy response. DNA from 19 TNBC patients undergoing neoadjuvant therapy were subjected to next generation sequencing of a panel of 24 genes in DNA repair and breast cancer predisposition. In this study, 5 of 19 patients (26%) carried a pathogenic mutation in BRCA1, PALB2, RAD51C and two patients carried a probable pathogenic missense variant. Moreover, VUS (Variants of Unknown Significance) in other genes, predicted to be deleterious by in silico tools, were detected in five patients. Germline mutations in DNA repair genes were found to be associated with the group of TNBC patients who responded to therapy. We conclude that a subgroup of TNBC patients have defects in DNA repair genes, other than BRCA1, and such patients respond favourably to neoadjuvant anthracyclines/taxanes therapy. © 2016 Wiley Periodicals, Inc.
三阴性乳腺癌(TNBC)约占所有乳腺癌病例的15 - 20%,其特征是具有复杂的分子异质性。一些TNBC表现出与BRCA突变肿瘤相似的临床和病理特征,但实际上BRCA基因并无突变。这种“BRCA样”表型可能由参与DNA修复的其他基因的种系突变所解释。尽管它们对烷化剂化疗有反应,但复发和进展风险很高。一些研究显示了新辅助治疗对有DNA修复缺陷的TNBC患者的疗效,但仍需要合适的DNA修复缺陷生物标志物。在此,我们研究了DNA修复基因的突变是否可能与蒽环类/紫杉烷类新辅助治疗反应相关。对19例接受新辅助治疗的TNBC患者的DNA进行了一组24个DNA修复和乳腺癌易感性相关基因的二代测序。在本研究中,19例患者中有5例(26%)在BRCA1、PALB2、RAD51C中携带致病性突变,2例患者携带可能致病性错义变异。此外,在5例患者中检测到其他基因中的意义未明变异(VUS),这些变异经计算机工具预测为有害。发现DNA修复基因的种系突变与对治疗有反应的TNBC患者组相关。我们得出结论,除BRCA1外,一部分TNBC患者存在DNA修复基因缺陷,这类患者对新辅助蒽环类/紫杉烷类治疗反应良好。© 2016威利期刊公司