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Background. The histopathological classification and staging system for uterine sarcoma (US) were revised in 2003 and 2009, respectively. However, there is currently no consensus on the significance of various prognostic factors. Therefore the available clinicopathological data on US are summarized in this review. Methods. Articles on uterine sarcoma published in English from 1970 to 2011 were identified systematically by computer-based searches in Medline and the Cochrane Library. Results. Prognosis of US is poor, with a five-year survival rate as low as 30%. The most common histological types are leiomyosarcoma (LMS, 63%), endometrial stromal sarcoma (ESS, 21%), adenosarcoma (6%), undifferentiated sarcoma (5%) and other types (5%). Carcinosarcoma is a mixed tumor, which is today regarded as a subset of endometrial carcinoma. Disease stage is the most important prognostic factor for all types of US. However, the prognosis of stage I LMS is also significantly related to tumor size and mitotic index (MI), and stage I ESS is related to MI and tumor cell necrosis (TCN). In adenosarcoma, TCN is the only significant histopathological prognostic factor. Information on the use of preoperative imaging for staging purposes is lacking. Total hysterectomy is the cornerstone of US treatment. The ovary can be preserved in premenopausal women with early-stage LMS and ESS, and routine lymphadenectomy is not necessary unless enlarged lymph nodes are present. As tumor-free resection margins at primary surgery are the most important prognostic factor for survival, sarcoma surgery should be centralized. Adjuvant treatment has changed from radiation therapy to chemotherapy over the last decades, without any change in survival. Conclusion. There are differences in survival between histological types of US. LMS and ESS can be divided into different prognostic groups and should be treated separately. [ABSTRACT FROM AUTHOR]