Uterine sarcomas have been known for the poor prognosis and high mortality rate. In addition, since uterine sarcomas are a rare group and heterogenous group of tumors with many pathologic subtypes, it is difficult to define optimal management in patients with uterine sarcoma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy have been considered as the cornerstone of treatment for patients with uterine sarcoma, although the efficacy of adnexectomy or lymphadenectomy is still controversial in several subtypes. Also, the role of adjuvant therapy including chemotherapy or radiotherapy remains unclear, although many studies support a possible benefit in terms of progression-free interval or pelvic control. In advanced uterine sarcoma, through the clinical trial, doxorubicin for leimyosarcoma and ifosfamide alone or with combination cisplatin for mixed mesodermal sarcoma represent reasonable therapeutic options. Recently gemcitabine combined with docetaxel has shown promising results for patients with leiomyosarcoma and there are currently some ongoing randomized clinical trials through the Gynecology Oncology Group. In conclusions, multi-institution, randomized clinical trials by histological stratification or accounting to the difference in natural history are more needed actively in patients with uterine sarcoma.ope
