Uterine Carcinosarcoma

Uterine carcinosarcomas are diagnosed in less than five percent of women with a uterine malignancy, with an annual incidence in the United States approximating 1-4 per 100,000 women. The pathogenesis of carcinosarcoma is poorly understood. In the multiclonal theory, both components have distinct cells of origin, whereas the tumor arises from a single multipotent stem cell that differentiates along epithelial and mesenchymal pathways in the monoclonal theory. Surgery is the mainstay of treatment for the majority of women with uterine carcinosarcoma. For women without clinical evidence of extrauterine disease, comprehensive surgical staging should be performed to ensure appropriate staging. Adjuvant medical therapy is routinely recommended for women with carcinosarcoma, regardless of stage. However, for women with early-stage disease, there is little evidence that adjuvant treatment improves overall survival, although studies have shown that treatment can reduce the risk of recurrence and improve progression-free survival.