ABSTRACT
Abstract Ovarian sex cord-stromal tumors (SCSTs) are a heterogeneous group o f neoplasms
that develop from the intraovarian matrix. These tumors account for less than 5% o f all ovarian malignancies and may develop at any age. SCSTs account for nearly 90% o f all functioning ovarian neoplasms. Patients often present with clinical manifestations o f excessive estrogen or androgen production. The scarcity o f these tumors limits the understanding o f their natural history, management and prognosis. Ovarian SCSTs exhibit indolent growth and either behave in a clinically benign fashion or have low malignant potential. Complete surgical resection is the mainstay o f treatment. The vast majority o f SCSTs are confined to one ovary at presentation, and few patients will require postoperative therapy. Recurrent disease is relatively insensitive to chemo therapy, but patients often live for many years due to slow tumor progression. The overall prognosis o f ovarian SC STs is excellent-primarily due to early diagnosis and curative surgery.