Chemotherapy of ovarian germ cell tumors and sex cord stromal tumors.

Abstract

Malignant ovarian germ cell tumors (OGCT) and sex cord stromal tumors (SCST) are much less common than epithelial ovarian cancer, each accounting for less than 5% of all ovarian malignancies. The combination of vincristine, dactinomycin, and cyclophosphamide (VAC) became the standard chemotherapy for patients with OGCT in the 1970s; it produced excellent sustained remission rates in patients with stage I disease but less than 50% sustained remission rates in those with metastatic tumor. With the introduction of cisplatin for the treatment of testicular cancer in the late 1970s, platinum-based regimens replaced the VAC regimen by the mid-1980s. Currently, the most popular regimen for all patients with OGCT is the combination of bleomycin, etoposide, and cisplatin (BEP). The BEP regimen appears to be superior to VAC, with sustained remission rates of more than 75% in patients with metastatic tumor. For patients with metastatic pure dysgerminoma, chemotherapy appears to have supplanted radiotherapy as standard treatment with the advantage of preserving fertility in most patients. For patients with SCST, no standard therapy exists. Surgery alone is currently acceptable treatment for all patients with SCST except those with metastatic disease, sarcomas, or Sertoli-Leydig cell tumors with poor differentiation or heterologous elements. Currently, platinum-based combination chemotherapy is favored for these latter patients but activity with such regimens is only modest.