Endometrial carcinosarcoma Review


Authors: Bogani, G.; Ray-Coquard, I.; Concin, N.; Ngoi, N. Y. L.; Morice, P.; Caruso, G.; Enomoto, T.; Takehara, K.; Denys, H.; Lorusso, D.; Coleman, R.; Vaughan, M. M.; Takano, M.; Provencher, D. M.; Sagae, S.; Wimberger, P.; Póka, R.; Segev, Y.; Kim, S. I.; Kim, J. W.; Candido dos Reis, F. J.; Ramirez, P. T.; Mariani, A.; Leitao, M.; Makker, V.; Abu-Rustum, N. R.; Vergote, I.; Zannoni, G.; Tan, D.; McCormack, M.; Paolini, B.; Bini, M.; Raspagliesi, F.; Benedetti Panici, P.; Di Donato, V.; Muzii, L.; Colombo, N.; Pignata, S.; Scambia, G.; Monk, B. J.
Review Title: Endometrial carcinosarcoma
Abstract: Endometrial carcinosarcoma is a rare and aggressive high-grade endometrial carcinoma with secondary sarcomatous trans-differentiation (conversion theory). The clinical presentation and diagnostic work-up roughly align with those of the more common endometrioid counterpart, although endometrial carcinosarcoma is more frequently diagnosed at an advanced stage. Endometrial carcinosarcoma is not a single entity but encompasses different histological subtypes, depending on the type of carcinomatous and sarcomatous elements. The majority of endometrial carcinosarcomas are characterized by p53 abnormalities. The proportion of POLE and microsatellite instablity-high (MSI-H) is directly related to the epithelial component, being approximately 25% and 3% in endometrioid and non-endometrioid components. The management of non-metastatic disease is based on a multimodal approach with optimal surgery followed by (concomitant or sequential) chemotherapy and radiotherapy, even for early stages. Palliative chemotherapy is recommended in the metastatic or recurrent setting, with carboplatin/paclitaxel doublet being the first-line regimen. Although the introduction of immunotherapy plus/minus a tyrosine kinase inhibitor shifted the paradigm of treatment of patients with recurrent endometrial cancer, patients with endometrial carcinosarcoma were excluded from most studies evaluating single-agent immunotherapy or the combination. However, the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) approved the use of pembrolizumab and lenvatinib in endometrial cancer (all histotypes) after progression on chemotherapy and single-agent immunotherapy in MSI-H cancers. In the era of precision medicine, emerging knowledge on molecular endometrial carcinosarcoma is opening new promising therapeutic options for more personalized treatment. The present review outlines state-of-the-art knowledge and future directions for patients with endometrial carcinosarcoma. © 2023 IGCS and ESGO.
Keywords: multimodality cancer therapy; combined modality therapy; endometrial neoplasms; carboplatin; neoplasm recurrence, local; pathology; genital neoplasms, female; tumor recurrence; uterus cancer; uterine neoplasms; endometrium tumor; uterine cancer; carcinosarcoma; humans; human; female
Journal Title: International Journal of Gynecological Cancer
Volume: 33
Issue: 2
ISSN: 1048-891X
Publisher: Lippincott Williams & Wilkins  
Date Published: 2023-02-01
Start Page: 147
End Page: 174
Language: English
DOI: 10.1136/ijgc-2022-004073
PUBMED: 36585027
PROVIDER: scopus
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Source: Scopus
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  1. Vicky Makker
    263 Makker
  2. Mario Leitao
    575 Leitao