Journal
CRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY
Volume 163, Issue -, Pages -Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.critrevonc.2021.103369
Keywords
Uterine carcinosarcoma; Malignant mixed Mullerian tumor; Epithelial-to-mesenchymal transition; Biphasic tumor
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Uterine carcinosarcoma, also known as malignant mixed M & uuml;llerian tumor, is a rare gynecological malignancy with poor prognosis. Surgical resection is the best curative option for localized disease, and additional peri-operative treatments have been shown to improve outcomes. Palliative chemotherapy is the treatment of choice for metastatic disease, with no consensus on the best regimen.
Uterine carcinosarcoma (UCS), also known as malignant mixed M & uuml;llerian tumor, is a rare gynecological malignancy characterized by poor prognosis. This biphasic neoplasm presents an admixture of epithelial and mesenchymal/sarcomatoid tumor cells which partially share their molecular signature and exhibit a typical epithelial-to-mesenchymal transition gene expression profile. Due to the rarity of this cancer, at present there is a scarcity of specific treatment guidelines. Surgical resection remains the best curative option for localized disease, whereas the addition of peri-operative radiotherapy, chemotherapy and chemoradiation has been shown to further improve disease outcomes. In the metastatic setting, palliative chemotherapy is currently the treatment of choice, although no consensus exists about the best regimen to be delivered. Besides standard treatment options for the advanced disease, mechanistic insights into UCS pathogenesis and identification of its histopathological and molecular features boosted the development of novel, and potentially more effective, therapeutic agents, that will be here discussed.
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