Management of recurrent juvenile granulosa cell tumor of the ovary.

Background: Juvenile granulosa cell tumors of the ovary are a rare form of neoplasm that makes up less than 5% of ovarian tumors in childhood and adolescence. About 90% are diagnosed in stage I with a favorable prognosis. More advanced stages (FIGO stages II--IV) have a poor prognosis.

Case: A patient was initially diagnosed at age 17 with FIGO stage IIIC disease and treated with a right salpingo-oophorectomy, debulking, and staging followed by six cycles of carboplatin and etoposide chemotherapy. Tumor recurrence in the liver and adjacent to the spleen occurred 13 months after completion of primary therapy. Aggressive surgical removal of tumor followed by six cycles of bleomycin and taxol as salvage chemotherapy resulted in 44 months of disease-free survival. On November 27, 2000, she had a cesarean delivery of a 2335-g normal male due to a breech presentation. Exploration revealed no evidence of tumor.

Conclusion: This is the second case report of a patient with advanced juvenile granulosa cell tumor to become pregnant after apparently successful chemotherapy. These results are encouraging, but the best treatment for extensive and recurrent disease has yet to be determined.