Variant classification changes over time in BRCA1 and BRCA2

Genet Med. 2019 Oct;21(10):2248-2254. doi: 10.1038/s41436-019-0493-2. Epub 2019 Apr 11.

Abstract

Purpose: To report BRCA1 and BRCA2 (BRCA1/2) variant reassessments and reclassifications between 2012 and 2017 at the Advanced Molecular Diagnostics Laboratory (AMDL) in Toronto, Canada, which provides BRCA1/2 testing for patients in Ontario, and to compare AMDL variant classifications with submissions in ClinVar.

Methods: Variants were assessed using a standardized variant assessment tool based on the American College of Medical Genetics and Genomics/Association for Molecular Pathology's guidelines and tracked in an in-house database. Variants were shared through the Canadian Open Genetics Repository and submitted to ClinVar for comparison against other laboratories.

Results: AMDL identified 1209 BRCA1/2 variants between 2012 and 2017. During this period, 32.9% (398/1209) of variants were reassessed and 12.4% (150/1209) were reclassified. The majority of reclassified variants were downgraded (112/150, 74.7%). Of the reclassified variants, 63.3% (95/150) were reclassified to benign, 20.7% (31/150) to likely benign, 10.0% (15/150) to variant of uncertain significance, 2.0% (3/150) to likely pathogenic, and 4.0% (6/150) to pathogenic. Discordant ClinVar submissions were found for 40.4% (488/1209) of variants.

Conclusion: BRCA1/2 variants may be reclassified over time. Reclassification presents ethical and practical challenges related to recontacting patients. Data sharing is essential to improve variant interpretation, to help patients receive appropriate care based on their genetic results.

Keywords: BRCA1; BRCA2; genetic testing; variant reclassification.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • BRCA1 Protein / classification
  • BRCA1 Protein / genetics*
  • BRCA2 Protein / classification
  • BRCA2 Protein / genetics*
  • Breast Neoplasms / classification
  • Breast Neoplasms / genetics*
  • Databases, Genetic
  • Female
  • Genetic Predisposition to Disease
  • Genetic Testing / methods
  • Genetic Testing / standards
  • Genetic Variation / genetics
  • Genomics
  • Humans
  • Information Dissemination

Substances

  • BRCA1 Protein
  • BRCA1 protein, human
  • BRCA2 Protein
  • BRCA2 protein, human

Grants and funding