Management of advanced uterine leiomyosarcoma

Purpose of review The purpose of this article is to review current evidence-based management strategies for patients with recurrent and metastatic uterine leiomyosarcoma (LMS). We will focus on treatment of advanced multifocal disease as well as new developments in targeted cancer therapies. Recent findings The management of patients with advanced uterine LMS is divided between those with localized and those with disseminated disease. Selected patients with localized or single-organ oligometastatic disease may benefit from surgical resection. For patients with disseminated disease, fixed-dose-rate gemcitabine plus docetaxel is an appropriate first-line chemotherapy regimen. Other active cytotoxic agents include doxorubicin, ifosfamide, and dacarbazine. The role of trabectedin (approved by the European Medicine Agency to be marketed for advanced or metastatic soft tissue sarcoma) is being explored. Trials are also underway for targeted therapy in uterine LMS. Currently, the only approved targeted therapy for advanced soft tissue sarcoma is pazopanib. In patients with small volume and slowly progressive estrogen receptor/progesterone receptor-positive disease, antiestrogen therapy with an aromatase inhibitor is a reasonable alternative to observation alone. Summary Despite recent advances, overall survival for advanced disease remains poor and identification of novel agents with activity in LMS is clearly needed.