The tumors typically produce androgens, and clinical virilization is noted in 70% to 85% of patients (449). Signs of virilization include oligomenorrhea followed by amenorrhea, breast atrophy, acne, hirsutism, clitoromegaly, deepening of the voice, and a receding hairline. Measurement of plasma androgens may reveal elevated testosterone and androstenedione, with normal or slightly elevated dehydroepiandrosterone sulphate (2,450). Rarely, the Sertoli-Leydig tumor is associated with manifestations of estrogenization (i.e., isosexual precocity, irregular or post-menopausal bleeding).
Because these low-grade lesions are only rarely bilateral (<1%), the usual treatment is unilateral salpingo-oophorectomy and evaluation of the contralateral ovary for patients who are in their reproductive years (3,422). For older patients, hysterectomy and bilateral salpingo-oophorectomy are appropriate (447,448).
There are insufficient data to document the utility of radiation or chemotherapy for patients with persistent disease, but some responses in patients with measurable disease were reported with pelvic irradiation and the VAC chemotherapy regimen (3,451–455).
The 5-year survival rate is 70% to 90%, and recurrences thereafter are uncommon (3,450– 455). Most fatalities occur in the presence of poorly differentiated tumors.
Figure 37.24 Sertoli-Leydig cell tumor of the ovary. Note the aggregates of eosinophilic Leydig cells in the stroma adjacent to Sertoli cell tubules.
Lipoid Cell Tumors Lipoid cell tumors are believed to arise in adrenal cortical rests that reside in the vicinity of the ovary. More than 100 cases were reported, and bilateral disease was noted in only a few (2). Most are associated with virilization and, occasionally, with obesity, hypertension, and glucose intolerance, reﬂecting corticosteroid secretion. Rare cases of estrogen secretion and isosexual precocity were reported.
Most of these tumors have benign or low-grade behavior, but about 20%, most of which are initially larger than 8 cm in diameter, are associated with metastatic lesions. Metastases are usually in the peritoneal cavity but can occur at distant sites. The primary treatment is surgical extirpation of the primary lesion. There are no data regarding the effectiveness of radiation or chemotherapy for this disease.
Sarcomas Malignant mixed mesodermal tumors (MMMT) of the ovary or carcinosarcomas are uncommon. Clinical, morphologic and molecular data suggest that MMMTs are monoclonal and are metaplastic carcinomas in which the mesenchymal part reﬂects dedifferentiation. Most tumors are heterologous, and 80% occur in postmenopausal women. The signs and symptoms are similar to those of most ovarian malignancies. These tumors are biologically aggressive, and most patients have evidence of metastases at presentation. They are treated either with carboplatin and paclitaxel or iphosphamide and cisplatin, but in general have a poor prognosis.
Small Cell Carcinoma, Hypercalcemic Type
This rare tumor occurs at an average age of 24 years (range 2 to 46 years) (456). The tumors are typically unilateral. Approximately two-thirds of the tumors are accompanied by paraendocrine hypercalcemia. This tumor accounts for one-half of all of the cases of hypercalcemia associated with ovarian tumors. About 50% of the tumors have spread beyond the ovaries when they are diagnosed (456). Immunohistochemical stains are helpful to differentiate this tumor from a lymphoma, leukemia, or sarcoma.