Hormone therapy in ovarian granulosa cell tumors: a systematic review.

OBJECTIVE This systematic review assessed the effectiveness of hormone therapy (HT) in patients with a granulosa cell tumor (GCT) of the ovary. METHODS Medline (OVID), EMBASE (OVID), the Cochrane Central Register of Controlled Trials (CENTRAL), prospective trial registers and PubMed (as supplied by publisher-subset) were searched up to January 13, 2014. No restrictions were applied. Two reviewers independently screened studies for eligibility and extracted data using a standardized, piloted extraction form. Studies evaluating the response to hormone therapy in patients with a GCT were included. The primary outcome was the objective response rate (ORR) to hormone therapy. RESULTS In total, nineteen studies including 31 patients were eligible. Pooled ORR to hormone therapy was 71.0% (95% Confidence Interval 52-85). In 25.8% a complete response and in 45.2% a partial response was described. Four patients had stable disease. In five patients disease was progressive. Various hormone treatments showed different results, for instance aromatase inhibitors (AI) demonstrated response in nine out of nine therapies (100%) and tamoxifen in none out of three (0%). Median progression free survival (PFS) after the start of hormone therapy was 18 months (range 0-60). CONCLUSIONS Despite the limited available data, hormone therapy appears to be a good treatment alternative for patients with advanced-stage or recurrent GCT. However, study quality is poor and prospective studies are needed to confirm clinical benefit of hormone therapy in GCTs.