Malignant salivary gland tumours in families with breast cancer susceptibility

Virchows Arch. 2021 Jul;479(1):221-226. doi: 10.1007/s00428-021-03105-6. Epub 2021 Jun 8.

Abstract

Salivary gland cancers (SGCs) are rare malignancies with highly heterogeneous histological features. Patients affected with SGCs are at increased risk of secondary malignancies, including breast cancer (BC). Previous studies enlightened a possible link between SGCs and hereditary predisposition to BC. Here, we searched for SGC-affected patients in 1796 high-risk BC families recruited at the Genetic Unit of the Istituto Nazionale dei Tumori of Milan, 516 of which carried pathogenic variants in BRCA1 and/or BRCA2, the main genetic risk factors for BC. We detected five families with an individual affected with SGC, including two male patients, one carrying a constitutional mutation in BRCA1 and the other in BRCA2. Loss of heterozygosity of BRCA wild-type alleles was assessed in the patients' tumour DNA. We conclude that our observations support the hypothesis that genetic factors associated with BC susceptibility might play a role also in at least a subset of SGCs.

Keywords: BRCA1; BRCA2; Loss of heterozygosity; Salivary gland cancer.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • BRCA1 Protein / genetics*
  • BRCA2 Protein / genetics*
  • Biomarkers, Tumor / genetics*
  • Breast Neoplasms / genetics*
  • Databases, Factual
  • Female
  • Genetic Predisposition to Disease
  • Heredity
  • Humans
  • Italy
  • Loss of Heterozygosity
  • Male
  • Middle Aged
  • Mutation*
  • Pedigree
  • Salivary Gland Neoplasms / genetics*

Substances

  • BRCA1 Protein
  • BRCA1 protein, human
  • BRCA2 Protein
  • BRCA2 protein, human
  • Biomarkers, Tumor