ABSTRACT

Sex cord-stromal tumors account for approximately 7% of all malignant ovarian neoplasms and their extreme rarity represents a limitation in our understanding of the natural history, management, and prognosis. Sex cord-stromal tumors of the ovary are derived from the sex cords and the ovarian stroma or mesenchyme. This category of ovarian neoplasms usually is composed of various combinations of elements, including the ‘‘female’’ cells (granulosa cells, theca cells, and their luteinized derivatives), ‘‘male’’ cells (Sertoli cells and Leydig cells), fibroblasts of gonadal-stromal origin as well as morphologically indifferent cells. A classification of this group of tumors is presented in Table 1.