Rare uterine cancer: carcinosarcomas. Review from histology to treatment

Uterine carcinosarcoma (UCS) is an aggressive malignancy. With an incidence of 2/100,000 females and a 5 years Survival at stage IV of 0%, it is an uncommon type of cancer with a very poor prognosis. Histologically, UCS is a biphasic neoplasm consisting of a mixture of malignant epithelial and mesenchymal components but there is now enough clinical-pathological evidence to consider UCS as metaplastic carcinoma in which the mesenchymal part retains epithelial features. The principal treatment in early/locally-advanced UCS is surgery; because of its aggressiveness, it generally presents distant metastases at diagnosis. Adjuvant radiotherapy and chemotherapy have uncertain effect. Chemotherapy alone or associated with radiotherapy seems to improve disease free survival (DFS) and overall survival (OS) in stage III and IV UCS. No advantages in OS and DFS have been shown with radiotherapy alone. The present review summarizes and analyzes the most important news about this type of gynaecological cancer.