Tumor stage is the strongest prognostic factor for all uterine sarcomas with 5-year survival of about 50–55 % for stage I and 8–12 % for more advanced stages [6]. When adjusted for stage, the prognosis of leiomyosarcoma is poorer than that of carcinosarcoma, but no survival difference is found between leiomyosarcoma and undifferentiated endometrial sarcoma. The prognosis of stage I leiomyosarcoma is also associated with the mitotic index (MI) and tumor size and of stage I endometrial stromal sarcoma with MI and tumor cell necrosis (TCN) [8]. Age, tumor grade, vascular space invasion, and p53, p16, and Ki-67 overexpression have been found to be prognostic variables in leiomyosarcoma in some studies [9–11], while DNA diploidy, S Phase fraction (SPF) less than 10 %, and progesterone receptor positivity seem to be associated with better outcomes [10, 12]. In endometrial stromal sarcomas, tumor-free resection margins were found to be the most important prognostic factor (P < 0.001) on a multivariate analysis, followed by tumor grade (P = 0.002), tumor diameter (P = 0.019), and menopausal status (P = 0.019) [13]. In carcinosarcomas, the role of pathological variables like cell type, lymph-node status, grade of epithelial component, grade and mitotic count of sarcomatous component, depth of myometrial invasion, lymph-vascular space involvement, and peritoneal cytology has been debated [2, 14]. Carcinosarcomas containing serous and clear cell carcinomas and heterologous components have been associated with poorer prognosis and survival rates [2, 15, 16]. Elevated postoperative serum CA-125 is also an independent prognostic factor for poor survival (HR 9.85; P < 0.001) [17] in these tumors. Tumor extent, vascular invasion, and nuclear uniformity are important prognostic variables for undifferentiated endometrial sarcomas [18, 19]. The prognosis of adenosarcoma is usually favorable, with features like extrauterine spread, deep myometrial invasion, and sarcomatous overgrowth (presence of pure sarcoma in more than 25 % of the tumor) associated with an increased risk of recurrence [4].

Total hysterectomy with bilateral salpingo-oophorectomy is the mainstay of treatment for uterine sarcomas. The ovaries can be preserved in premenopausal women with stage I leiomyosarcomas and endometrial stromal sarcomas. Routine lymphadenectomy is not necessary unless enlarged lymph nodes are present. The status of tumor-free resection margins at primary surgery is important for survival, and adjuvant therapy includes chemotherapy, radiotherapy, and hormone therapy (for ESS). Carcinosarcomas of the uterus should undergo full surgical staging including peritoneal washings, total hysterectomy with bilateral salpingo-oophorectomy, bilateral pelvic and para-aortic node dissection, and omental biopsy (or omentectomy) with excision of all gross disease. Adjuvant treatment includes radiotherapy depending on operative findings and chemotherapy.