Optimal management of uterine leiomyosarcoma.

Uterine leiomyosarcomas (LMSs) are rare tumors, comprising 1.3% of all uterine cancers. Primary therapy for localized disease entails complete surgical resection. The majority of patients recur within 2 years of primary therapy as these tumors tend to undergo early hematogenous spread. A randomized, controlled trial showed no improvement in the overall or disease-free survival with adjuvant radiotherapy, compared with observation, following resection of early-stage uterine LMS. A Phase II study of adjuvant chemotherapy following complete surgical resection of uterine LMS reported promising results. However, in the absence of Phase III randomized data demonstrating improved outcomes, the role of post-resection chemotherapy for early-stage disease remains experimental. For metastatic or unresectable LMS, systemic chemotherapy forms the mainstay of treatment. First-line treatment options include gemcitabine-docetaxel or doxorubicin with or without ifosfamide. Novel targeted therapies are under investigation in an attempt to devise more effective treatment strategies.