Uterine sarcomas are rare, high-risk malignancies. Expert histologic review is important for accurate diagnosis. For high-grade leiomyosarcomas, the risk of recurrence is high after complete resection of uterus-limited disease; however, no adjuvant therapy has been proven to improve survival. Chemotherapy regimens with efficacy in treating advanced uterine leiomyosarcoma include gemcitabine-docetaxel, doxorubicin and ifosfamide. Uterine carcinosarcomas also carry a high risk of recurrence. Adjuvant chemotherapy is a standard approach for completely resected and metastatic carcinosarcoma. Active agents include carboplatin, cisplatin, ifosfamide and paclitaxel.
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