Adult granulosa cell tumours of the ovary

Purpose of review Adult ovarian granulosa cell tumours (AGCTs) are the most common sex cord-stromal tumours. Although the prognosis is generally favourable, recurrent or advanced AGCT shows poor prognosis. An overview of the main findings on the management of AGCT published recently is provided. Recent findings Novel biomarkers, including FOXL2, SMAD3 and GATA4, have been identified as potential diagnostic and therapeutic targets for this type of tumour. Interesting therapeutic implications are also emerging from studies on preclinical models, supporting the possible activity of anti-vascular endothelial growth factor A therapy for the treatment of AGCTs. Further, potentially active drugs could be targeting agents directed against epidermal growth factor receptor and/or insulin growth factor receptor-1. Recent data confirmed the importance of surgery in the management of AGCTs, in which hysterectomy can be avoided in young patients, as a recent study demonstrated that the risk of endometrial cancer after salpingo-oophorectomy for AGCT, with negative endometrial evaluation, is lower than the risk of endometrial cancer in the general population. Summary The present review highlights current challenges and future directions in the treatment of AGCTs. Optimization of existing treatment modalities and the addition of novel drugs may hopefully lead to improved oncologic outcomes.