Abstract
Uterine leiomyosarcoma (LMS) is a rare, but clinically aggressive smooth muscle malignancy. Although uterine malignancies such as endometrial cancer are common, uterine LMS accounts for only 1% of uterine cancers. Patients typically present with vaginal bleeding, pain and a high pelvic mass. Treatment for uterine LMS includes hysterectomy. Uterine LMS is histologically characterized by nuclear atypia, mitotic figures and coagulative necrosis. Surgical staging and grade are valid prognostic indicators for survival. However, in the absence of metastatic disease or enlarged lymph nodes, there is no evidence supporting the survival benefit of bilateral salpingo-oophorectomy or lymphadenectomy. Although adjuvant radiation reduces the risk of pelvic relapse, it does not improve overall survival. Additionally, there is no evidence supporting adjuvant chemotherapy to improve survival. The combination of gemcitabine plus docetaxel demonstrates significant response rates for both advanced and metastatic disease. Secondary cytoreductive surgery appears to be beneficial in a select group of patients with recurrent uterine LMS. Despite modest improvements in treatment, recurrence rates remain high, and 5-year survival rates remain low. Continued investigation into traditional therapies, such as surgery and chemotherapy, as well as novel approaches, including those based on molecular biology for uterine LMS, are needed. Contemporary management strategies for uterine smooth muscle tumors and uterine LMS will be presented.