Ovarian Sex Cord-Stromal Tumors

Abstract

Ovarian sex cord-stromal tumors are clinically significant heterogeneous tumors that include several pathologic types. These tumors are often found in adolescents and young adults and can present with hormonal manifestations as well as signs and symptoms of a pelvic mass. Serum tumor markers may assist in preoperative diagnosis and surveillance. Several subtypes are associated with genetic predisposition, including those observed in patients with Peutz-Jegher syndrome. Recent studies have elucidated the relationship between Sertoli-Leydig cell tumors and DICER1 mutations. When classified as International Federation of Gynecology and Obstetrics stage Ia, most subtypes may be treated with surgery alone. Higher stage or recurrent tumors have variable prognoses that range from a usually rapid course in poorly differentiated Sertoli-Leydig cell tumor to an often prolonged course in adult granulosa cell tumors. New understanding of the molecular pathogenesis of these tumors may pave the way for novel therapeutics.

FIG 1.

Common histologic features of juvenile granulosa cell tumors, Sertoli-Leydig tumors, and gynandroblastoma ovarian neoplasms. (A and B) Low-power and high-power views of juvenile granulosa cell tumor showing nodular arrangement of tumor cells in pale blue background with mucoid pools. Cells have ovoid, regular nuclei and ill-defined cytoplasm. (C and D) Sertoli-Leydig tumors have multiple histologic patterns. Panel C shows tubular arrangements of Sertoli cells with hyperchromatic nuclei and high nuclear-cytoplasmic ratio. An anaplastic mitotic figure is visible on the right side of the photomicrograph. Panel D shows the typical nested arrangement of Sertoli cells admixed with larger Leydig cells with abundant pink cytoplasm. (E and F) Gynandroblastomas feature a combination of juvenile granulosa cell tumor (E) and Sertoli-Leydig cell tumor (F) features. These two photomicrographs are from different areas of the same tumor.