Abstract: Uterine sarcomas account for less than 10% of all uterine neoplasms (Tropé et al., 2012). The most common include uterine leiomyosarcoma and the endometrial stromal neoplasms. The diagnosis requires pathologic review of the uterus in order to characterize extent of myometrial invasion. However, molecular diagnosis has aided the classification of endometrial stromal neoplasms, especially in helping to discriminate between endometrial stromal and undifferentiated endometrial sarcoma. The prognosis of these tumors following surgery varies, with endometrial stromal sarcoma associated with a better prognosis compared to leiomyosarcoma or undifferentiated endometrial sarcoma. For aggressive sarcomas, there is interest in adjuvant treatment, which has focused on the evaluation of systemic agents. However, the rarity of these tumors makes the conduct of prospective trials difficult and no consensus adjuvant regimen has emerged. In the absence of Level I data, the use of chemotherapy is based on institutional preferences. Ongoing clinical trials will help inform the standard treatment approach for these tumors, and we encourage patients with uterine sarcoma to participate in well-designed clinical trials.