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Endometrial carcinosarcoma
  1. Giorgio Bogani1,
  2. Isabelle Ray-Coquard2,
  3. Nicole Concin3,
  4. Natalie Yan Li Ngoi4,
  5. Philippe Morice5,
  6. Giuseppe Caruso6,
  7. Takayuki Enomoto7,
  8. Kazuhiro Takehara8,
  9. Hannelore Denys9,
  10. Domenica Lorusso10,
  11. Robert Coleman11,
  12. Michelle M Vaughan12,
  13. Masashi Takano13,
  14. Diane Michele Provencher14,
  15. Satoru Sagae15,
  16. Pauline Wimberger16,
  17. Robert Póka17,
  18. Yakir Segev18,
  19. Se Ik Kim19,
  20. Jae-Weon Kim19,
  21. Francisco Jose Candido dos Reis20,
  22. Pedro T Ramirez11,
  23. Andrea Mariani21,
  24. Mario Leitao22,
  25. Vicky Makker22,
  26. Nadeem R Abu-Rustum23,
  27. Ignace Vergote24,
  28. Gianfranco Zannoni25,
  29. David Tan4,
  30. Mary McCormack26,
  31. Biagio Paolini27,
  32. Marta Bini28,
  33. Francesco Raspagliesi28,
  34. Pierluigi Benedetti Panici29,
  35. Violante Di Donato29,
  36. Ludovico Muzii30,
  37. Nicoletta Colombo31,
  38. Sandro Pignata32,
  39. Giovanni Scambia33 and
  40. Bradley J Monk34
  1. 1Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
  2. 2Centre Leon Berard, LYON CEDEX 08, Centre, France
  3. 3Department of Gynecology and Obstetrics; Innsbruck Medical Univeristy, Innsbruck, Austria
  4. 4National University Cancer Institute, Singapore
  5. 5Department of Surgery, Institut Gustave RoussT, Villejuif, France
  6. 6Department of Maternal and Child Health and Urological Sciences, Policlinico Umberto I, Rome, Italy
  7. 7Department of Obstetrics and Gynecology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Belgium
  8. 8Department of Gynecologic Oncology, National Hospital Organization Shikoku Cancer Center, Matsuyama, Japan
  9. 9Department of Medical Oncology, University Hospital Ghent, Gent, Belgium
  10. 10Policlinico Gemelli, Rome, Italy
  11. 11Department of Gynecologic Oncology and Reproductive Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
  12. 12Department of Medical Oncology, Canterbury Regional Cancer and Haematology Service, Christchurch, New Zealand
  13. 13Department of Obstetrics and Gynecology, National Defense Medical College, Tokorozawa, Medical, Japan
  14. 14Centre Hospitalier de LUniversite de Montreal, Montreal, Québec, Canada
  15. 15Hokkaido Ohno Memorial Hospital, Sapporo, Japan
  16. 16Department of Gynecology and Obstetrics, Technische Universitat Dresden Medizinische Fakultat Carl Gustav Carus, Dresden, Germany
  17. 17University of Debrecen, Debrecen, Hungary
  18. 18Department of Obstetrics and Gynecology, Carmel Hospital, Haifa, Israel
  19. 19Department of Obstetrics and Gynecology, Seoul National University Hospital, Seoul, Korea
  20. 20Ginecologia e Obstetricia, Universidade de Sao Paulo Faculdade de Medicina de Ribeirao Preto, Ribeirao Preto, Brazil
  21. 21Department of Gynecologic Surgery, Mayo Clinic Rochester, Rochester, Minnesota, USA
  22. 22Memorial Sloan-Kettering Cancer Center, New York, New York, USA
  23. 23Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York, USA
  24. 24Department of Gynecology and Obstetrics, Gynecologic Oncology, Leuven Cancer Institute, Catholic University Leuven, Leuven, Belgium
  25. 25Dipartimento Scienze della Salute della Donna e del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
  26. 26Department of Oncology, University College London Hospitals NHS Foundation Trust, London, UK
  27. 27Istituto Nazionale per lo Studio e la Cura dei Tumori, Milano, Italy
  28. 28Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Lombardia, Italy
  29. 29Department of Obstetrics and Gynecology, University Sapienza of Roma, Rome, Italy
  30. 30Department of Maternal, Infantile, and Urological Sciences, Umberto I Hospital, Sapienza University of Rome, Roma, Italy
  31. 31Medical Gynecologic Oncology Unit; University of Milan Bicocca; Milan; Italy, European Institute of Oncology, Milano, Italy
  32. 32Department of Gynaecological Oncology, National Cancer Institute Napels, Naples, Italy
  33. 33Dipartimento Scienze della Salute della Donna e del Bambino, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy
  34. 34HonorHealth, University of Arizona, Creighton University, Phoenix, Arizona, USA
  1. Correspondence to Dr Giorgio Bogani, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano 20133, Italy; giorgiobogani{at}yahoo.it

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.

  • uterine cancer
  • carcinosarcoma
  • genital neoplasms, female

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Footnotes

  • Twitter @DenysHannelore, @pedroramirezMD, @leitaomd

  • Contributors Substantial contributions to the conception or design of the work: All authors. Drafting the work or revising it critically for important intellectual content: All authors. Final approval of the version to be published: All authors. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: All authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests GB: Novartis AG Pharma (C/A, H), Italian Ministry of Health (RG); NC: AstraZeneca (C/A, SH), Seattle Genetics (C/A, SH), MSD (SAB), Mersana (C/A, SH), eTheRNA immunotherapies NV (C/A, SH), Roche (travel expenses), Genmab (travel expenses), Amgen (travel expenses). IR-C: honoraria from AstraZeneca, Clovis, GSK/Tesaro, and PharmaMar; consulting/advisory board fees from AstraZeneca, Roche, Clovis, GSK/Tesaro, Genmab, PharmaMar, MSD, Mersana, Deciphera, OncXea, Esai, BMS, Novartis, and Pfizer; research funding from MSD; travel expenses from AstraZeneca, GSK, and Roche. YS: AstraZeneca (CA), GSK (CA). PW: Amgen (SH, RF, SAB), AstraZeneca (SH, RF, H, SAB), Clovis (SH, RF, SAB), Eisai (SH, SAB), GSK (SH, SAB), Lilly (SH, SAB), MSD (SH, RF, SAB), Novartis (SH, RF, SAB), Pfizer (SH, RF, SAB), Roche (SH, RF, H, SAB), TEVA (SH, SAB). NYLN: AstraZeneca (SH), Janssen (SH). KT: AstraZeneca (SH), Chugai (SH, RF), Eisai (SH), MSD (SH), Mochida (SH), Takeda (SH). TE: Takeda (SH), Astra Zeneca (SH), Eisai (SH), Chugai Pharma (SH, RF), MSD (SH), Mochida (SH). DL: AstraZeneca (H, CA), Clovis (H, CA, RF), GSK/Tesaro (H, CA), Roche (CA), Genmab (CA), PharmaMar (CA, RF), MSD (CA, RF), Esai (CA), Merck Serono (CA), Novartis (CA), and PharmaMar (H); consulting/advisory board fees from AstraZeneca, Roche, Clovis, GSK/Tesaro. DMP: AstraZeneca (CA, SH, SAB), GSK (CA, SH, SAB). NRA-R: NIH/NCI Cancer Center support grant P30 CA008748 (F). HD: Roche (CA, SH, SAB), Pfizer (CA, SH, SAB), AstraZeneca (SH, SAB), Lily (SAB), GSK (SAB), Novartis (SH), Pharmamar (SH); BJM: AstraZeneca (SH, SAB), GSK (SH, SAB), Incyte (SAB), Merck (SH, SAB), Roche/Genentech (SH, SAB), Eisai (SAB), GOG-Foundation (E), US Oncology (E).

  • Provenance and peer review Not commissioned; externally peer reviewed.