Recurrent ovarian granulosa cell tumor: role of combination chemotherapy with report of a long-term response to a cyclophosphamide, doxorubicin and cisplatin regimen.

We report a patient who, at age 43, underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy for an unrupted 10 cm granulosa cell tumor. A recurrence was subtotally totally resected 2.5 years later, followed by six cycles of cyclophosphamide, doxorubicin, and cis-platin (CAP) chemotherapy. She had no evidence of disease at second-look laparotomy. Serum estradiol (E2) levels paralleled her clinical course, becoming elevated at the time of her recurrence, and returning to postmenopausal levels during her chemotherapy. Four years later, further elevation in E2 heralded a second recurrence of tumor. The patient underwent a cytoreductive procedure and has resumed chemotherapy. Reports of the few other patients treated with multiagent chemotherapy are reviewed. Several combinations appear active, with the CAP regimen having possibly less toxicity. Compared with radiotherapy, chemotherapy may yield longer survival in patients with recurrent granulosa cell tumor, but actual cure remains elusive.