Uterine sarcomas are a group of rare and usually aggressive soft tissue cancers. They have a wide range of histologic appearances, from myomatous to osteous to stromal. The three major subtypes of uterine sarcomas (listed in decreasing order of incidence) are carcinosarcoma, leiomyosarcoma, and endometrial stromal sarcoma. Most patients with uterine sarcomas are middle-to older-aged women who present with abnormal uterine bleeding or pelvic mass, which may be confused with leiomyoma. Surgery - including hysterectomy and resection of disease - serves as the main treatment modality. Adjuvant therapies, including radiation, chemotherapy, and/or hormonal therapy, have limited benefit on overall survival; however, this may be due to the lack of good randomized controlled trials of sufficient size because of uterine sarcomas' rare and aggressive nature. For patients with metastatic recurrent disease, aggressive therapy is limited by low response rates and limited duration of response. For patients with uterine sarcomas, enrollment in clinical trials is strongly encouraged.
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