Relative to her risk of breast carcinoma, the woman with a BRCA1 or BRCA2 g
ene mutation can be managed either by intensive screening (with or without
chemoprevention) or by prophylactic mastectomy. Although it would be prefer
able to avoid prophylactic surgery, the current level of screening technolo
gy and the rudimentary state of chemoprevention do not guarantee a good out
come with intensive surveillance. A review of the currently available data
was undertaken to determine the efficacy of prophylactic surgery, intensive
screening, and chemoprevention. An attempt then was made to extrapolate th
e efficacy of the Various approaches to the management of women who carry B
RCA1 or BRCA2 gene mutations. Intensive surveillance may not detect breast
carcinoma at an early, curable stage in young women with BRCA1 or BRCA2 gen
e mutations because the growth rate of the tumors in these women most likel
y will be rapid and the density of the breast tissue may compromise detecti
on. Chemoprevention is in its infancy, and its efficacy in this population
is unknown. Conversely, prophylactic surgery may not be completely effectiv
e in preventing breast carcinoma. The authors are hopeful that sometime in
the next decade advances in chemoprevention, screening technology, or breas
t carcinoma treatment will make mastectomy obsolete. However, for the time
being prophylactic mastectomy has attributes that make it an alternative fo
r this population that must be considered. Careful discussion of all option
s is essential in the management of these women. Cancer 1999;86:2502-16. (C
) 1999 American Cancer Society.