What is the risk of breast cancer in male carriers of pathogenic ATM mutations?

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Last updated: February 24, 2026View editorial policy

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Male Breast Cancer Risk in ATM Mutation Carriers

Male carriers of pathogenic ATM mutations have a modestly increased risk of breast cancer with an odds ratio of approximately 3.4, though the absolute lifetime risk remains low at less than 2%. 1

Quantified Risk Estimates

The evidence for male breast cancer risk in ATM mutation carriers is limited but consistent:

  • A recent study identified ATM pathogenic variants in 4 out of 340 BRCA1/2-negative male breast cancer cases, generating an odds ratio of 3.36 (95% CI 0.89-8.96, P=0.04). 1

  • An earlier Greek study found that approximately 2% of males with breast cancer harbor pathogenic variants in ATM. 1

  • Among 627,742 patients referred for multigene panel testing including 4,607 ATM heterozygotes, male breast cancer showed an odds ratio of 1.72 (95% CI 1.08-2.75). 2

Context and Comparison to Other Genes

The risk for male breast cancer in ATM carriers is substantially lower than in BRCA2 carriers:

  • Male BRCA2 carriers have a cumulative lifetime risk of 7-8% for breast cancer. 1

  • Male BRCA1 carriers have a cumulative lifetime risk of 1.2% for breast cancer. 1

  • In contrast, men without BRCA1/2 mutations have a baseline lifetime risk of approximately 0.1% (1 in 1,000). 1

Strength of Evidence

The evidence for increased male breast cancer risk in ATM carriers is characterized as "weak" by the most recent ACMG clinical practice resource (2025). 1 This reflects:

  • Small absolute numbers of cases in published studies
  • Wide confidence intervals that sometimes cross 1.0
  • Limited prospective data
  • Most evidence comes from case-control studies rather than prospective cohort studies

Clinical Implications

Annual mammography or ultrasound screening should be considered in male ATM carriers only if they have additional high-risk features such as gynecomastia or Klinefelter syndrome, starting from age 50 or 10 years before the earliest male breast cancer in the family. 1

Male ATM carriers should be encouraged to be aware of physical changes in the breast and seek medical attention for any breast masses, skin changes, or nipple discharge. 1

Important Caveats

  • The risk estimates are primarily derived from populations of European descent, and may not be generalizable to other ethnic groups. 1

  • Unlike female breast cancer risk in ATM carriers (which is well-established at approximately 2-fold increased risk), the male breast cancer association remains less certain due to the rarity of male breast cancer overall. 1

  • Routine screening mammography is not recommended for male ATM carriers without additional risk factors, as there is little evidence of efficacy. 1

References

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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