The treatment of uterine sarcomas

Abstract

Uterine sarcomas are rare and comprise only 3% of all uterine cancers. Within the group of adult soft tissue sarcomas, they account for ∼7% of new cases. They consist of several distinct histological subtypes like leiomyosarcoma, endometrial stromal tumors, undifferentiated sarcomas, pure heterologous sarcomas, and mixed epithelial and mesenchymal tumors. Standard treatment in localized disease is abdominal hysterectomy. Bilateral salpingo-oophorectomy and lymphadenectomy have no proven value in leiomyosarcomas and high-grade undifferentiated sarcomas. However, in endometrial stromal tumors, given the hormonal reponsiveness of most tumors, salpingo-oophorectomy is generally recommended. Carcinosarcomas are treated according to current recommendations for epithelial uterine cancers. In leiomyosarcomas, postoperative radiation does not improve both relapse-free and overall survival. adjuvant chemotherapy seems to improve survival in the context of uncontrolled phase II trials. However, it is currently not considered standard of care in the absence of data from randomized trials. In contrast, adjuvant chemotherapy does improve overall survival in carcinosarcomas and is therefore considered standard of care. Systemic therapy for advanced uterine leiomyosarcomas, undifferentiated uterine sarcomas, and heterologous sarcomas is generally following the recommendations for adult soft tissue sarcomas. Endometrial stromal sarcomas are usually hormonal receptor positive, which allows endocrine therapy in most cases.