BRCA1 hereditary breast and ovarian cancer syndrome

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Disease Overview

BRCA1 hereditary breast and ovarian cancer syndrome (BRCA1 HBOC) is an inherited condition that is characterized by an increased risk for a variety of different cancers. Women with this condition have a 57-60% risk of developing breast cancer, a 40-59% risk of developing ovarian cancer and an 83% risk of developing contralateral breast cancer by age 70. Men have a 1% lifetime risk of breast cancer and an increased risk for prostate cancer. BRCA1 HBOC may also be associated with an elevated risk for cancers of the cervix, uterus, pancreas, esophagus, stomach, fallopian tube, and primary peritoneum; however, these risks are not well defined.[8477][8479] This condition is caused by changes (mutations) in the BRCA1 gene and is inherited in an autosomal dominant manner.[8480] Management may include high risk cancer screening, chemoprevention and/or prophylactic surgeries.[8477][8481]


Synonyms

  • BREAST CANCER, FAMILIAL, SUSCEPTIBILITY TO, 1, INCLUDED
  • OVARIAN CANCER, FAMILIAL, SUSCEPTIBILITY TO, 1, INCLUDED
  • Familial susceptibility to breast-ovarian cancer 1
  • BROVCA1

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National Organization for Rare Disorders