Ovarian Sex Cord-Stromal Tumors

Abstract

Sex cord-stromal tumors represent the third group of ovarian tumors in decreasing order of frequency, with approximately 5–10 % of cases falling within this category. The most common entity among these tumors—ovarian fibroma—is readily recognized on frozen sections by a general surgical pathologist; however, other sex cord-stromal tumors may present a significant challenge due to their diverse morphology. In addition, unlike in other tumor types, the biological behavior of sex cord-stromal tumors is usually not directly linked to their microscopic degree of pleomorphism or cytological atypia. As an example, most adult granulosa cell tumors have uniform nuclei and relatively low mitotic activity, despite their locally aggressive behavior and overall 20–30 % recurrence rate. Similarly, the nuclear atypia may not be obvious in juvenile granulosa cell tumors and in biologically aggressive forms of Sertoli-Leydig cell tumors. While histological subtyping of primary ovarian epithelial malignancies on frozen section is typically not necessary and may be deferred for permanent sections, mere identification of an ovarian tumor as in origin, without recognition of the specific entity, is not sufficient for predicting outcome and guiding intraoperative management.