Uterine sarcomas comprise a group of rare tumors with different tumor biology, natural history and response to treatment, contain just 3-7% of total uterine malignancies and about 1% of all gynecologic cancers. Although they cause important part of women death due gynecologic cancers. These tumors have aggressive behavior and high recurrence rate, even when confined to the uterine corpus at the time of diagnosis. The most common of uterine sarcomas is leiomyosarcoma. The incidence of leiomyosarcoma is increased after age 50. Traditionally, carcinosarcomas were named as Malignant Mixed Mullerian tumor (MMMT), but in recent classification according to their pathologic structure and its behavior, these tumors are classified as carcinomas. The rare group of sarcomas is endometrial stromal sarcoma (ESS), which occurres in younger women. In a medical studies search from 2000 to 2017, all kinds of uterine sarcomas, pathologic diagnostic methods, primary treatment and supportive treatment have been analyzed. Last histological classification is based on FIGO 2009 and WHO. According to such classification, sarcomas divided into three subtypes: leiomyosarcoma, endometrial stromal sarcoma and carcinosarcomas. Diagnosis of sarcoma before treatment and discrimination from benign myoma by current diagnostic methods is difficult. Preoperative endometrial sampling identifies only 25% of sarcomas. It may be the myometrial origin of tumor. Currently, MRI, ultrasound and PET scan may be used for the diagnosis of tumor. The gold standard of treatment is complete and intact resection of tumor considereing free margins. In advanced or recurrence disease, cytoreductive surgery followed by chemotherapy is the choice of treatment. If technically it is not possible or there are extra abdominal metastases, palliative chemotherapy should be considered. Combination of gemcitabine and docetaxel is an acceptable choice. Recent studies are going to approve the effective role for targeted agents with or without cytotoxic chemotherapy in these group of aggressive tumors. The only drug in this group has approval is pazopanib. However, it did not achieved acceptable responses in phase I, II studies. As regards of tumor biology and inappropriate response to chemotherapy and radiotherapy, sarcoma have poor prognosis in all stages.
