Department of Genetics, University Medical Center Utrecht, PO Box 85090, 3508 AB, Utrecht, The Netherlands.
Family Cancer Clinic, Netherlands Cancer Institute, Amsterdam, The Netherlands.
Fam Cancer. 2019 Apr;18(2):231-239. doi: 10.1007/s10689-018-0103-5.
Germline TP53 mutations are associated with an increased risk of early-onset breast cancer. Traditionally, it was not standard practice to offer TP53 genetic testing due to the low mutation detection rate and limited options regarding preventive screening. Recent guidelines recommend that all women diagnosed with breast cancer before the age of 31, irrespective of family history, should be offered TP53 genetic testing. This study aims to gain more knowledge on the attitudes and experiences among genetics professionals regarding the timing and content of genetic counselling of young breast cancer patients for Li-Fraumeni syndrome (LFS). We conducted a nationwide online survey among genetics professionals who provide cancer genetic counselling in the Netherlands. Fifty-seven professionals completed the questionnaire (response rate overall 54%, clinical geneticists 70%). Most respondents reported that they discuss the option of TP53 genetic testing-simultaneously with BRCA 1/2-during the initial counselling visit, especially in case of referral for treatment-focused genetic counselling. There was a general consensus about ten information items that should be discussed during counselling. Sixty-one percent of genetics professionals did not encounter difficulties in providing genetic counselling for LFS, but a substantial minority (29%) did. This study offers valuable insight, which will be useful for clinical practice. Studies which address young breast cancer patients' attitudes and preferences regarding the timing and content of counselling are warranted to further determine the most appropriate genetic counselling strategy for these women.
胚系 TP53 突变与早发性乳腺癌风险增加相关。传统上,由于突变检测率低且预防性筛查选择有限,因此不常规进行 TP53 基因检测。最近的指南建议,所有 31 岁以下确诊乳腺癌的女性,无论家族史如何,都应进行 TP53 基因检测。本研究旨在更多地了解遗传专业人员对年轻乳腺癌患者 Li-Fraumeni 综合征 (LFS) 遗传咨询时机和内容的态度和经验。我们在荷兰提供癌症遗传咨询的遗传专业人员中进行了一项全国性在线调查。57 名专业人员完成了问卷调查(总体回复率为 54%,临床遗传学家为 70%)。大多数受访者报告说,他们在初始咨询访问期间同时讨论 TP53 基因检测的选项——与 BRCA 1/2 一起,特别是在转诊进行治疗为重点的遗传咨询的情况下。在咨询期间应该讨论十个信息项目方面存在普遍共识。61%的遗传专业人员在为 LFS 提供遗传咨询方面没有遇到困难,但相当一部分(29%)确实遇到了困难。这项研究提供了有价值的见解,这将对临床实践很有用。需要进行研究以进一步确定这些女性最合适的遗传咨询策略,以确定年轻乳腺癌患者对咨询时机和内容的态度和偏好。