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.