General Medical Information

Uncommon Ovarian Cancers

Jonathan S. Berek and Neville F. Hacker

There are several varieties of malignant ovarian tumors, which together comprise only 0.1% of ovarian malignancies. These lesions include lipoid (or lipid) cell tumors, primary ovarian sarcomas, and small cell ovarian carcinomas.

Lipoid Cell Tumors
Lipoid cell tumors are thought to arise in adrenal cortical rests that reside in the vicinity of the ovary. More than 100 cases have been reported, and bilaterality has been noted in only a few. Most are associated with virilization, and occasionally with obesity, hypertension, and glucose intolerance reflecting glucocorticoid secretion. Rare cases of estrogen secretion and isosexual precocity have been reported.

Most of these tumors have a benign or low-grade behavior, but approximately 20%, most of which are initially larger than 8 cm in diameter, produce metastatic lesions. Metastases are usually in the peritoneal cavity but occur rarely at distant sites. The primary treatment is surgical extirpation of the primary lesion. There are no data regarding radiation or chemotherapy for this disease.

Sarcomas
Malignant mixed mesodermal sarcomas of the ovary are extremely rare; only approximately 100 cases have been reported. Most lesions are heterologous, and 80% occur in postmenopausal women. The presentation is similar to that of most ovarian malignancies. These lesions are biologically aggressive, and most patients have evidence of metastases.

Such patients should be treated by cytoreductive surgery and postoperative platinum-containing combination chemotherapy. In patients in whom all macroscopic disease can be resected, we have observed disease-free survival of more than 3 years in two patients treated with six cycles of cisplatin and epirubicin.

Small Cell Carcinomas
This rare tumor occurs at an average age of 24 years (range, 2 to 46 years). The tumors are all bilateral. Approximately two thirds of the tumors are accompanied by paraendocrine hypercalcemia. This tumor accounts for one half of all cases of hypercalcemia associated with ovarian tumors. Approximately 50% of the tumors have spread beyond the ovaries when they are diagnosed.

The management of these malignancies consists of surgery followed by platinum-based chemotherapy or radiation therapy. In addition to the primary treatment of the disease, control of the hypercalcemia may require aggressive hydration, loop diuretics, and the use of bisphosphonates or calcitonin. The prognosis tends to be poor, with most patients dying within 2 years of diagnosis in spite of treatment.