Abstract

Background

Unbiased estimates of penetrance are challenging but critically important to make informed choices about strategies for risk management through increased surveillance and risk-reducing interventions.

Methods

We studied the penetrance and clinical outcomes of 7 breast cancer susceptibility genes (BRCA1, BRCA2, TP53, CHEK2, ATM, PALB2, and PTEN) in almost 13 458 participants unselected for personal or family history of breast cancer. We identified 242 female participants with pathogenic or likely pathogenic variants in 1 of the 7 genes for penetrance analyses, and 147 women did not previously know their genetic results.

Results

Out of the 147 women, 32 women were diagnosed with breast cancer at an average age of 52.8 years. Estimated penetrance by age 60 years ranged from 17.8% to 43.8%, depending on the gene. In clinical-impact analysis, 42.3% (95% confidence interval = 31.3% to 53.3%) of women had taken actions related to their genetic results, and 2 new breast cancer cases were identified within the first 12 months after genetic results disclosure.

Conclusions

Our study provides population-based penetrance estimates for the understudied genes CHEK2, ATM, and PALB2 and highlights the importance of using unselected populations for penetrance studies. It also demonstrates the potential clinical impact of genetic testing to improve health care through early diagnosis and preventative screening.

Details

Title
Penetrance of Breast Cancer Susceptibility Genes From the eMERGE III Network
Author
Fan, Xiao 1 ; Wynn, Julia 2 ; Shang, Ning 3 ; Liu, Cong 3 ; Fedotov, Alexander 4 ; Hallquist, Miranda L G 5 ; Buchanan, Adam H 5 ; Williams, Marc S 5 ; Smith, Maureen E 6 ; Hoell, Christin 7 ; Rasmussen-Torvik, Laura J 8 ; Peterson, Josh F 9 ; Wiesner, Georgia L 10 ; Murad, Andrea M 11 ; Jarvik, Gail P 12 ; Gordon, Adam S 7 ; Rosenthal, Elisabeth A 12 ; Stanaway, Ian B 12 ; Crosslin, David R 13 ; Larson, Eric B 14 ; Leppig, Kathleen A 14 ; Henrikson, Nora B 14 ; Williams, Janet L 5 ; Li, Rongling 15 ; Hebbring, Scott 16 ; Weng, Chunhua 3 ; Shen, Yufeng 17 ; Crew, Katherine D 18 ; Chung, Wendy K 19 

 Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, USA; Department of Systems Biology, Columbia University Irving Medical Center, New York, NY, USA 
 Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, USA 
 Department of Biomedical Informatics, Columbia University Irving Medical Center, New York, NY, USA 
 Irving Institute for Clinical and Translational Research, Columbia University Irving Medical Center, New York, NY, USA 
 Genomic Medicine Institute, Geisinger, Danville, PA, USA 
 Department of Medicine, Northwestern University, Chicago Feinberg School of Medicine, Chicago, IL, USA 
 Center for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA 
 Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA 
 Department of Biomedical Informatics, Vanderbilt University Medical Center, Nashville, TN, USA 
10  Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA 
11  Division of Genetic Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA 
12  Department of Medicine (Medical Genetics), University of Washington Medical Center, Seattle, WA, USA 
13  Department of Biomedical Informatics and Medical Education, University of Washington Medical Center, Seattle, WA, USA 
14  Kaiser Permanente Washington Health Research Institute, Seattle, WA, USA 
15  Division of Genomic Medicine, National Human Genome Research Institute, National Institutes of Health, Baltimore, MD, USA 
16  Center for Precision Medicine Research, Marshfield Clinic, Marshfield, WI, USA 
17  Department of Systems Biology, Columbia University Irving Medical Center, New York, NY, USA; Department of Biomedical Informatics, Columbia University Irving Medical Center, New York, NY, USA 
18  Herbert Irving Comprehensive Cancer Center, Columbia University Irving Medical Center, New York, NY, USA; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA 
19  Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, USA; Herbert Irving Comprehensive Cancer Center, Columbia University Irving Medical Center, New York, NY, USA; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA 
Publication year
2021
Publication date
Aug 2021
Publisher
Oxford University Press
e-ISSN
25155091
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3170591769
Copyright
© The Author(s) 2021. Published by Oxford University Press. This work is published under http://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.