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World Journal of Urology

, Volume 37, Issue 1, pp 115–123 | Cite as

Epidemiology, biology and treatment of sarcomatoid RCC: current state of the art

  • Cedric LebacleEmail author
  • Aydin Pooli
  • Thomas Bessede
  • Jacques Irani
  • Allan J. Pantuck
  • Alexandra Drakaki
Invited Review
  • 243 Downloads

Abstract

Long recognized to confer an extremely poor prognosis, sarcomatoid dedifferentiation of renal cell carcinoma (sRCC) is a tumor phenotype that is finally beginning to be better understood on the molecular and genetic levels. With an overall incidence that ranges from 1 to 32% depending on associated RCC subtype, the survival of sarcomatoid RCC patients rarely exceeds 2 years. The main reasons for its poor outcome include its aggressive biology, its tendency to present at an advanced or metastatic stage at the time of diagnosis, its high rate of tumor recurrence after nephrectomy, and its limited response to systemic therapies. Molecular pathology studies suggest that sarcomatoid dedifferentiation originates from a focal epithelial–mesenchymal transition (EMT) arising in the carcinomatous component of the tumor. It is hoped that the growing understanding of the molecular biology of sRCC will soon make it possible to adapt treatments based on the identification of actionable tumor alterations. The deliberate inclusion of these patients in the multicenter clinical trials of immune, targeted and combination therapies is a necessary next step in pioneering future treatment strategies.

Keywords

Sarcomatoid renal cell carcinoma Kidney neoplasms Immunotherapy 

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of UrologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  2. 2.Institute of Urologic Oncology (IUO)David Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of UrologyUniversity Hospital Bicetre, APHPLe Kremlin BicetreFrance
  4. 4.Department of Hematology and OncologyDavid Geffen School of Medicine at University of CaliforniaLos AngelesUSA

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