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.