Introduction Hormone-producing tumors of the ovary are extremely uncommon and are thus unfamiliar to most practicing gynecologists. Because of this it would seem ideal to organize this review by presenting signs and symptoms. Unfortunately, patients may have similar tumors but very different clinical manifestations depending on their age. Furthermore, many tumors may produce either estrogens or androgens. Therefore, for clarity, we have chosen to organize the review by histopathology, placing all tumors into either sex cord-stromal or germ cell classification. A summary organized by tumor type is presented in Table 1 and by reproductive status and presentation in Table 2.

Diagnosis of hormone-secreting tumors is usually straightforward as most ovarian masses can be palpated during pelvic examination. Additionally, vaginal ultrasound can be very helpful in identifying very small, non-palpable ovarian masses. For extra-ovarian masses, computed tomography or magnetic resonance imaging can detect very small lesions. Rarely, ovarian tumors