Double CHEK2 Pathogenic and Low-Risk Variants and Associated Cancer Phenotypes
2025

Double CHEK2 Variants and Cancer Risk

Sample size: 3783 publication Evidence: moderate

Author Information

Author(s): Bychkovsky Brittany L. MD MSc, Agaoglu Nihat B. MD PhD, Horton Carolyn MS, Polfus Linda PhD, Richardson Marcy E. PhD, Young Colin PhD, Scheib Rochelle MD, Garber Judy E. MD MPH, Rana Huma Q. MD MPH

Primary Institution: Dana-Farber Cancer Institute

Hypothesis

Are CHEK2 low-risk (LR) variants p.I157T, p.S428F, and p.T476M associated with cancer phenotype when in a biallelic state similar to biallelic pathogenic and likely pathogenic variants (PVs) in CHEK2?

Conclusion

Individuals with 2 LR variants in CHEK2 had a cancer phenotype similar to those with a single LR variant and similar to wild-type controls.

Supporting Evidence

  • Individuals with 2 LR variants had a cancer prevalence of 76.9%, similar to wild-type controls.
  • 95.0% of individuals with 1 PV and 1 LR variant had a prior cancer diagnosis.
  • Individuals with 2 LR variants had a similar cancer phenotype to those with a single LR variant.

Takeaway

This study looked at how certain gene changes might affect cancer risk. It found that having two specific low-risk gene changes doesn't seem to increase cancer risk more than having one.

Methodology

This retrospective observational cohort study examined cancer phenotype by CHEK2 genotype in individuals who underwent genetic testing.

Potential Biases

The study population is racially homogenous, predominantly White.

Limitations

The study had relatively few individuals with biallelic CHEK2 variants and was subject to bias as cases were ascertained through clinical cancer genetic testing.

Participant Demographics

92.1% were female, median age at testing was 53 years, and included various racial and ethnic backgrounds.

Digital Object Identifier (DOI)

10.1001/jamanetworkopen.2024.51361

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