Background. Genetics plays a role in all cancers. Evidence exists for
the presence of inherited genes associated with the development of ova
rian cancer in three familial ovarian cancer syndromes: a site-specifi
c ovarian cancer syndrome, a breast/ovarian cancer syndrome, and an ov
arian cancer syndrome associated with hereditary nonpolyposis colorect
al cancer. Methods and Results. The authors present an updated summary
of recent advances within the field of ovarian cancer genetics and ex
amine the extent to which this genetic information, at both an epidemi
ologic and molecular level, may be used to identify a subset of women
who are likely to be at increased risk of developing ovarian cancer. I
n addition, the extent to which these data may be used to define metho
ds of prevention or treatment for women at risk is discussed. Conclusi
on. Women who are members of high risk ovarian cancer families should
receive genetic screening and medical follow-up in an effort to reduce
their overall chances of morbidity and death associated with the deve
lopment of ovarian and other cancers. The construction of cancer famil
y registries will help to identify women at risk and facilitate their
entry into clinical trials and screening programs for ovarian cancer.