Ovarian cancer represents the fifth most significant cause of cancer-related death for women and is the most frequent cause of death from gynecological neoplasia in the Western world. The incidence of ovarian cancer in the United Kingdom (U.K.) is over 5000 new cases every year, accounting for 4275 deaths per year (1 ). The lifetime risk of ovarian cancer for women in the U.K. is approximately 1 in 80. Most (80-90%) ovarian tumors are epithelial in origin and arise from the coelomic epithelium. The remainder arise from germ-cell or sex cord/stromal cells. A hereditary component in the latter group is rare, but includes granulosa-cell tumors in patients with PeutzJeghers syndrome (2 ) and autosomal dominant inheritance of small-cell carcinoma of the ovary (3 ,4 ). Because of their limited contribution to familial ovarian cancer, these nonepithelial tumors will not be considered further in this chapter.
