Ovarian Sex Cord Stromal Tumours | Chris O'Brien Lifehouse

General Considerations

Are uncommon tumours, accounting for less than 5% of all ovarian malignancies. They arise from cells derived from the sex cords or mesenchyme of the embryonic gonad and contain granulosa cells, Sertoli cells, Leydig cells, thecal cells or fibroblasts of gonadal stromal origin either singly or in combination. They are classified according to their differentiation toward “female directed cells” classified as granulosa-theca cell tumours or “male directed cells” or Sertoli Leydig cell tumors. Their malignant potential depends on the cell type. Granulosa cell tumours with or without a theca cell component are the commonest, and 15% of these are malignant. Thecomas are less common and usually benign. Thecomas and granulosa cell tumors produce estrogen. Sertoli-Leydig tumors (androblastomas) cause virilisation following defeminisation; they are the least common and of low-grade malignancy. Hormonal production is not consistent and has not proven to be a reliable means of classifying this group of neoplasms. Up to 15% are hormonally inert. Paradoxically tumours composed of female directed cells can produce androgens while tumors composed of male directed cells can produce oestrogens. Gynandroblastomas are composed of primitive mesenchymal cells of both female and male derivation (granulosa and Sertoli and Leydig elements). Granulosa cell tumours, thecomas, fibromas and Leydig cell tumours effect women usually in their 50’s. Sertoli-Leydig cell tumours occur in women in their 20-30’s and juvenile granulosa cell tumours in women under age 20. Due to their unopposed oestrogen production up to 50% of granulosa cell tumours have associated endometrial hyperplasia and 5-15% have will have an associated adenocarcinoma of the uterus. Characteristic histological features include Call Exner bodies in granulosa cell tumors and Reinke crystals in leydig cell tumors.

Treatment

In premenopausal women, an attempt to preserve fertility by performing a unilateral salpingoophorectomy and fractional D+C may be all that is required. In postmenopausal women, a THBSO is recommended (107, 108). Adjuvant therapy is not considered in Stage I disease. Exceptions to this may include rare variants such as grade 3 SLCT, heterologous and retiform SLCT, lipoid tumour, SCST with annular tubules and not associated with Peutz Jeghers syndrome, and fibrosarcoma). The appropriate treatment for advanced and recurrent disease may be either irradiation therapy or combination chemotherapy (109). While there is little data available, BEP, as used in germ cell tumours is recommended (110). Secondary cytoreduction may have a role if the disease appears localized, appears to be resectable and the disease free interval is greater than 12 months. Hormone levels can be used in surveillance for recurrence (111). Prognosis is excellent with greater than 90-95% 5-year survival, primarily due to early stage at diagnosis and low grade malignancy. Prognosis correlates with stage, tumor size, presence of rupture, grade, LVSI and mitotic rate (108).

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