Hormonal therapy in gynecological sarcomas

Abstract

Gynecological sarcomas are rare, constituting 3-5% of uterine malignancies. Endometrial stromal sarcomas and some uterine leiomyosarcomas are characterized by estrogen receptor (ER) and progesterone receptor (PgR) expression with variable impact on their clinical behavior and potential response to systemic therapies. A variety of hormonal treatments have been tested, since they act as targeted treatment against ER and PgR and have a tolerable side effect profile, which allows them to be administered for prolonged periods. Their role has been studied more extensively in endometrial stromal sarcomas, as the majority of cases are ER/PgR positive, while recently, an emerging role for hormonal manipulation has been described in ER/PgR-positive uterine leiomyosarcomas. Owing to the rarity and heterogeneous nature of uterine sarcomas, current treatment recommendations are based on small retrospective studies and case reports. This review comprises a critical appraisal of the existing data regarding hormonal manipulation in uterine sarcomas and attempts to make recommendations for endocrine treatments in specific settings, as well as suggest targets/medications for future research.