Dedifferentiation in renal cell carcinoma (RCC), either sarcomatoid or rhabdoid, is an infrequent event that may occur heterogeneously in the setting of any RCC histology and is associated with poor outcomes. Sarcomatoid dedifferentiation is associated with inferior survival with angiogenesis targeted therapy and infrequent responses to cytotoxic chemotherapy. However, immune checkpoint therapy has significantly improved outcomes for patients with sarcomatoid dedifferentiation. Biologically, sarcomatoid dedifferentiation has increased programmed death-ligand 1 (PD-L1) expression and an inflamed tumor microenvironment, in addition to other distinct molecular alterations. Less is known about rhabdoid dedifferentiation from either a clinical, biological, or therapeutic perspective. In this focused review, we will discuss the prognostic implications, outcomes with systemic therapy, and underlying biology in RCC with either sarcomatoid or rhabdoid dedifferentiation present.
Cancer treatment and research communications. 2022 Sep 22 [Epub ahead of print]
Andrew W Hahn, Justin Lebenthal, Giannicola Genovese, Kanishka Sircar, Nizar M Tannir, Pavlos Msaouel
Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America. Electronic address: ., Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America; Department of Genomic Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Department of Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America; Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America; Department of Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America. Electronic address: .