The treatment of advanced uterine
leiomyosarcomas (U-LMS) represents a considerable challenge. Radiological diagnosis prior to
hysterectomy is difficult, with the diagnosis frequently made postoperatively. Whilst a total abdominal
hysterectomy is the cornerstone of management of early disease, the role of routine adjuvant pelvic
radiotherapy and
adjuvant chemotherapy is less clear, since they may improve local
tumor control in high risk patients but are not associated with an overall survival benefit. For recurrent or disseminated U-LMS, cytotoxic
chemotherapy remains the mainstay of treatment. There have been few active
chemotherapy drugs approved for advanced disease, although newer drugs such as
trabectedin with its pleiotropic mechanism of actions represent an important addition to the standard front-line systemic
therapy with
doxorubicin and
ifosfamide. In this review, we outline the therapeutic potential and in particular the emerging evidence-based strategy of
therapy with
trabectedin in patients with advanced U-LMS.