TY - JOUR
T1 - Uterine carcinosarcoma: An overview.
AU - Pezzicoli, Gaetano
AU - Moscaritolo, Francesca
AU - Silvestris, Erica
AU - Silvestris, Franco
AU - Cormio, Gennaro
AU - Porta, Camillo
AU - D'Oronzo, Stella
N1 - Publisher Copyright:
© 2021 Elsevier B.V.
PY - 2021/5/27
Y1 - 2021/5/27
N2 - Uterine carcinosarcoma (UCS), also known as malignant mixed Müllerian 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.
AB - Uterine carcinosarcoma (UCS), also known as malignant mixed Müllerian 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.
KW - Biphasic tumor
KW - Epithelial-to-mesenchymal transition
KW - Malignant mixed Müllerian tumor
KW - Uterine carcinosarcoma
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U2 - 10.1016/j.critrevonc.2021.103369
DO - 10.1016/j.critrevonc.2021.103369
M3 - Review article
C2 - 34051304
AN - SCOPUS:85107152462
VL - 163
JO - Critical Reviews in Oncology/Hematology
JF - Critical Reviews in Oncology/Hematology
SN - 1040-8428
M1 - 103369
ER -