Sex cord stromal tumors of the ovary are rare neoplasms that account for only 7% of all ovarian malignancies. They are derived from the sex cords and the ovarian stroma or mesenchyme. Most common in this group is granulosa cell tumor that accounts for approximately 70% of malignant sex cord stromal tumors and divides into adult and juvenile type. Surgery remains the mainstay of initial management and is necessary to establish a definitive tissue diagnosis. In patients wishing to preserve their fertility unilateral salphingo-oophorectomy seems to be reasonable. In patients with advanced disease and in postmenopausal women total abdominal hysterectomy with bilateral salphingo-oophorectomy and complete surgical staging should be performed. Most of these tumors are classified as stage I at the time of diagnosis. Patients with stage I disease have a very good prognosis (5-years survival 84-95%) and do not require adjuvant treatment. Only long-term, careful follow-up is recommended. In advanced stages adjuvant chemotherapy with BEP is usually considered standard first line. Serum tumor markers are very useful in follow-up, especially inhibins and estradiol. Other sex cord stromal tumors include tumors from thecoma-fibroma group, Sertoli-Leydig cell tumors and very rare gynandroblastoma and sex cord tumors with annular tubules.

sex cord stromal tumors, granulosa cell tumor, thecoma-fibroma group, Sertoli-Leydig cell tumors, gynandroblastoma, sex cord tumors with annular tubules, management