Several androgen-secreting ovarian tumors can cause hir-sutism and virilization, including Sertoli-Leydig cell tumors and three rare neoplasms.
Sertoli–Leydig cell tumors (also called androblastoma andarrhenoblastoma) are ovarian neoplasms that secrete testosterone. These tumors constitute <0.4% of ovarian tumors and usu-ally occur in women between the ages of 20 and 40. Thetumor is most often unilateral (95% of cases) and may reach a size of 7 to 10 cm in diameter.
The history and physical examination give critical clues in diagnosing subjects who present with hirsutism and testosterone-secreting ovarian tumors.
Testosterone- secreting tumors usually have a more rapid onset and more severe hirsutism with virilizing signs. Women with aSertoli–Leydig cell tumor have a rapid onset of acne, hirsutism (75% of patients), amenorrhea (30% of patients), and viriliza-tion. A characteristic clinical course of two overlappingstages is described: first, the stage of defeminization, char-acterized by amenorrhea, breast atrophy, and loss of the subcutaneous fatty deposits responsible for the rounding of the feminine figure; and second, the stage of masculin-ization, characterized by clitoral hypertrophy, hirsutism, and deepening of the voice. These changes may occur over 6 months or less.
Laboratory studies of this disorder show suppression of FSH and LH, low plasma androstenedione, and marked elevation of testosterone. An ovarian mass may be palpable on pelvic examination. Once the diagnosis is suspected, there should be no delay in surgical removal of the involved ovary. The contralateral ovary should be inspected, and if it is found to be enlarged, it should be bisected for gross inspection.
Following surgical removal of a Sertoli–Leydig cell tumor, ovulatory cycles return spontaneously, and further progression of hirsutism is arrested. If the clitoris has become enlarged, it does not revert to its pretreatment size. However, temporal hair is restored, and the body habitus becomes feminine once again. Terminal hair in a sexual dis-tribution will not revert to vellus hair, but the growth and pigmentation will slow. Most patients will require mechan-ical removal of excess hair following removal of the ovarian tumor. The 10-year survival rates for this low-grade malignantovarian tumor approximate 90% to 95%.
Gynandroblastoma is a rare ovarian tumor, having bothgranulosa cell and arrhenoblastoma components. The predominant clinical feature is masculinization, although estrogen production may simultaneously produce endome-trial hyperplasia and irregular uterine bleeding.
Lipid (lipoid) cell tumors are usually small ovariantumors containing sheets of round, clear, pale-staining cells with a differential histologic diagnosis of hilar cell tumors, stromal luteoma of pregnancy, and Sertoli–Leydig cell tumors. The clinical presentation is masculinization or defeminization associated with elevated 17-ketosteroids in many cases.
Hilar cell tumors arise from an overgrowth of maturehilar cells or from ovarian mesenchyme and are typically found in postmenopausal women. They are characterized clinically by masculinization, which supports the idea that hilar cells are the homologs of the interstitial or Leydig cells of the testis. Histologically, the tumors contain pathognomonic Reinke albuminoid crystals in most cases, and grossly, they are always small, unilateral, and benign. Treatment is surgical removal for these three rare tumors.