The recent discovery of the breast cancer-associated genes BRCA1 and BRCA2
has changed the clinical care provided to women at high risk of boast cance
r. We will review what is currently known about the clinical management of
patients who bear (or are suspected of bearing) mutations in either of thes
e two genes. The issues related to establishing a diagnosis of inherited br
east cancer, deciding which women are candidates for testing, the limitatio
ns of testing, and the predictive power of these tests are addressed. The p
rognostic features of cancers associated with a BRCA1 and BRCA2 mutation ar
e reviewed. Further; guidelines for prophylaxis of women with a BRCA1 or BR
CA2 mutation are given and recommendations are made for the care of mutatio
n carriers with cancer. Semin. Surg. Oncol. 18:296-304, 2000, (C) 2000 Wile
y-Liss, Inc.