Ovarian neoplasms are the most frequent androgen-producing tumors. Granulosa cell tumors constitute 1% to 2% of all ovarian tumors and occur mostly in adult women (in postmenopausal more frequently than in premenopausal women) (see Chapter 37). Usually associated with estrogen production, they are the most common functioning tumors in children and can lead to isosexual precocious puberty (202). Patients can present with vaginal bleeding caused by endometrial hyperplasia or endometrial cancer resulting from prolonged exposure to tumor-derived estrogen (203). Total abdominal hysterectomy and bilateral salpingo-oophorectomy are the treatments of choice. If fertility is desired, a more conservative approach involving unilateral salpingo-oophorectomy with careful staging can be performed in women with stage IA (the cancer does not extend outside the involved ovary and a concomitant uterine cancer is excluded) (203). The malignant potential of these lesions is variable. The 10year survival rates vary from 60% to 90%, depending on the stage, tumor size, and histologic atypia (202).
Thecomas are rare and occur in older patients. In one study only 11% were androgenic, even in the presence of steroid-type cells (luteinized thecomas) (202). They are unilateral in more than 90% of the cases and rarely malignant. A unilateral oophorectomy is adequate treatment (204).
Sclerosing stromal tumors are benign neoplasms that usually occur in patients younger than 30 years (202). A few cases with estrogenic or androgenic manifestations were reported.
Sertoli-Leydig cell tumors, previously classified as androblastoma or arrhenoblastoma, account for 11% of solid ovarian tumors. They contain various proportions of Sertoli cells, Leydig cells, and fibroblasts (202). Sertoli-Leydig cell tumors are the most common virilizing tumors in women of reproductive age; however, masculinization occurs in only one-third of patients. The tumor is bilateral in 1.5%. In 80% of cases, it is diagnosed at stage IA (202). Sertoli-Leydig cell tumors are frequently low-grade malignancies, and their prognosis is related to their degree of differentiation and stage of disease (205). Treatment with unilateral salpingo-oophorectomy is justified in patients with stage IA disease who desire fertility. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, and adjuvant therapy are recommended for postmenopausal women who have advanced-stage disease.
Pure Sertoli cell tumors are usually unilateral. For a premenopausal woman with stage I disease, a unilateral salpingo-oophorectomy is the treatment of choice. Malignant tumors are rapidly fatal (206).
Gynandroblastomas are benign tumors with well-differentiated ovarian and testicular elements. A unilateral oophorectomy or salpingo-oophorectomy is sufficient treatment.
Sex cord tumors with annular tubules (SCTAT) are frequently associated with Peutz-Jeghers syndrome (gastrointestinal polyposis and mucocutaneous melanin pigmentation) (207). Their morphologic features range between those of the granulosa cell and Sertoli cell tumors.
Whereas SCTAT with Peutz-Jeghers syndrome tend to be bilateral and benign, SCTAT without Peutz-Jeghers syndrome is almost always unilateral and malignant in one-fifth of cases (202).