Uterine carcinosarcomas (MMMT—malignant mixed Müllerian tumours) are highly aggressive, rare, biphasic tumours composed of epithelial and mesenchymal elements believed to arise from a monoclonal origin. While hysterectomy with bilateral salpingo-oophorectomy remains the mainstay treatment, high rates of recurrence and metastases suggest a need for lymphadenectomy and postoperative adjuvant treatment. There are no established consensus guidelines for therapeutic patient management. Though well recognized that it improves locoregional control, the role of radiation in improving overall survival outcomes remains undecided. Although various combinations of chemotherapy have been explored, an optimal therapeutic modality is yet to be determined. As overall survival rates have not improved in thirty years, it is suggested that targeted chemotherapy and/or a multimodality approach may yield better outcomes. This paper provides a summary of the aetiopathogenesis of carcinosarcomas (MMMT) limited to the uterus with special emphasis on the controversies in the management of these patients. 1. Embryology and Historical Perspectives The name “malignant mixed Müllerian tumor” (MMMT) is derived from observations of the embryonic female genitalia. During the sixth week of embryogenesis, the Müllerian (paramesonephric) ducts created from intermediate mesoderm of the coelomic epithelium invaginate lateral to the mesonephric ducts. Epithelial and mesenchymal structures arise or are induced from the development of these Müllerian ducts [1]. In males, anti-Müllerian hormone secreted by the Sertoli cells of the testis causes rapid regression of these ducts; however, in females, this duct leads to the formation of the fallopian tubes, uterus, cervix, and cranial portion of the vagina. Certain Müllerian-type carcinomas have been identified, and metaplastic transformation of these carcinomas into sarcoma has been suggested on the basis of clonality analysis [2]. This is further supported by the finding that aside from the uterus, MMMTs have been identified, in decreasing order of frequency in the vagina [3], cervix [4], ovary [5], and most rarely the fallopian tube [6]. Additionally, on rare occasions, the female peritoneum can develop Müllerian-type neoplasms including MMMT [2]. For over 150 years, malignant neoplasms arising in the uterus composed of both epithelial and mesenchymal elements have been a subject of debate. Its origin dates back to 1852, wherein it was recognized as a mixed mesodermal tumour that was then called “enchondroma” [1]. Traditionally, MMMTs were
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