Department of Oncology, Biology and Genetics, University of Genoa, V.le Benedetto XV, 6, 16129, Genoa, Italy.
Fam Cancer. 2012 Mar;11(1):41-7. doi: 10.1007/s10689-011-9483-5.
Pancreatic adenocarcinoma (PC) is the third most common cancer associated with BRCA mutations. Most notice has been given to BRCA2, while the association between BRCA1 and PC is less widely reported. Recently, PALB2 has been implicated in both PC and breast cancer (BC) susceptibility. We selected 29 Italian PC patients from a case-control study of PC according to their personal and family history of both PC and breast/ovarian cancer (BC/OC) and tested them for presence of germline mutations in BRCA1, BRCA2 and PALB2. We identified no germline mutations or deletions in PALB2, but detected 7 BRCA mutations (4 in BRCA1 and 3 in BRCA2). These findings suggest that PALB2 does not play a major role in PC susceptibility in our population. As we found an almost equal frequency of germline mutations in BRCA1 and BRCA2, germline alterations in either of these genes may explain a subset of Italian families presenting both PC and BC/OC. Moreover, as we began the observation of these families from probands who are affected by PC, we provide here a direct assessment of the role of PALB2 and BRCA mutations in PC susceptibility.
胰腺导管腺癌(PC)是与 BRCA 突变相关的第三大常见癌症。大多数人都注意到了 BRCA2,而 BRCA1 与 PC 之间的关联则报道较少。最近,PALB2 与 PC 和乳腺癌(BC)易感性均有关联。我们根据个人和家族的 PC 和乳腺癌/卵巢癌(BC/OC)病史,从 PC 的病例对照研究中选择了 29 名意大利 PC 患者,并对他们的 BRCA1、BRCA2 和 PALB2 种系突变进行了检测。我们未在 PALB2 中发现种系突变或缺失,但在 BRCA1 中检测到 4 种突变,在 BRCA2 中检测到 3 种突变。这些发现表明 PALB2 并非我们人群中 PC 易感性的主要因素。由于我们在 BRCA1 和 BRCA2 中发现了几乎相同频率的种系突变,因此这些基因中的种系改变可能解释了存在 PC 和 BC/OC 的一部分意大利家族。此外,由于我们从患有 PC 的先证者开始观察这些家族,因此我们在此直接评估了 PALB2 和 BRCA 突变在 PC 易感性中的作用。