STROMAL TUMORS
Sex cord-stromal or sex cord-mesenchymal tumors include tumors of the female type (granulosa cell tumors and granulosa-theca cell tumors), the male type (Sertoli-Leydig tumors), and very rare types such as lipid cell tumors and gynandroblastoma. The majority of these rare tumors will never be seen by the practicing gynecologist.
Ovarian cancer
The granulosa cell tumor is the most common malignant tumor of this group of neoplasms. As shown in Figure 4, these tumors occur throughout a woman’s lifetime but are more common in the first four decades. They are bilateral in only about 5% of cases. All granulosa cell tumors should be considered potentially malignant, and late recurrences are common. There are two major histologic types: the adult variety and the juvenile form. The juvenile form is much more likely to result in a malignancy.
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The therapy of granulosa cell tumors is surgical removal; there is no proven benefit for adjunctive therapy. In younger patients who desire to retain their childbearing capability, unilateral salpingo-oophorectomy and full surgical staging are the treatment of choice. Patients with residual or recurrent disease should receive chemotherapy. The usual choice of chemotherapy is bleomycin, etoposide, and platinum, as it is in germ cell tumors. There are too few reported series of granulosa cell tumors treated with chemotherapy to provide accurate figures on response rates to chemotherapy.
Cancer treatment
Young patients with granulosa cell tumors may show precocious puberty because these tumors may produce estrogen. In older women, menstrual irregularity or post-menopausal bleeding due to estrogen production may be the presenting symptom.
Sertoli-Leydig tumors occur less frequently than granulosa cell tumors and are rarely bilateral. These tumors may produce androgens and can present clinically with defeminization or masculinization. The malignant potential of these tumors is directly related to the degree of differentiation, and they are usually classified as well differentiated, moderately differentiated, or poorly differentiated. A fourth classification is Sertoli-Leydig tumors with heterologous elements.
The treatment for these tumors is surgical removal, and unilateral salpingo-oophorectomy is indicated for patients with properly staged disease and tumors confined to one ovary. Little is known about the responsiveness of these tumors to chemotherapy, but most authors recommend bleomycin, etoposide, and platinum chemotherapy for persistent or recurrent disease.
Other stromal tumors are very rare. In general, the therapy is total abdominal hysterectomy and bilateral salpingo-oophorectomy, with full surgical staging for patients who do not desire further childbearing. For younger patients, unilateral salpingo-oophorectomy and full surgical staging are indicated.