The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma

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 target...

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Main Authors: Andrew W. Hahn, Justin Lebenthal, Giannicola Genovese, Kanishka Sircar, Nizar M. Tannir, Pavlos Msaouel
Format: Article
Language:English
Published: Elsevier 2022-01-01
Series:Cancer Treatment and Research Communications
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2468294222001319
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author Andrew W. Hahn
Justin Lebenthal
Giannicola Genovese
Kanishka Sircar
Nizar M. Tannir
Pavlos Msaouel
author_facet Andrew W. Hahn
Justin Lebenthal
Giannicola Genovese
Kanishka Sircar
Nizar M. Tannir
Pavlos Msaouel
author_sort Andrew W. Hahn
collection DOAJ
description 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.
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spelling doaj.art-c7b57c73b221438ca018feb03373a2d42022-12-22T03:00:15ZengElsevierCancer Treatment and Research Communications2468-29422022-01-0133100640The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinomaAndrew W. Hahn0Justin Lebenthal1Giannicola Genovese2Kanishka Sircar3Nizar M. Tannir4Pavlos Msaouel5Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America; Corresponding author at: Department of Genitourinary Medical Oncology, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd. Unit 1374, Houston, TX 77030.Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of AmericaDepartment 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 AmericaDepartment 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 AmericaDepartment of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of AmericaDepartment 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; Co-corresponding author at: Department of Genitourinary Medical Oncology, Division of Cancer Medicine, Department of Translational Molecular Pathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, 1155 Pressler St., Unit 1374, Houston, TX 77030-3721.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.http://www.sciencedirect.com/science/article/pii/S2468294222001319SarcomatoidRhabdoidDedifferentiationRenal cell carcinomaISUP gradeImmune checkpoint therapy
spellingShingle Andrew W. Hahn
Justin Lebenthal
Giannicola Genovese
Kanishka Sircar
Nizar M. Tannir
Pavlos Msaouel
The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
Cancer Treatment and Research Communications
Sarcomatoid
Rhabdoid
Dedifferentiation
Renal cell carcinoma
ISUP grade
Immune checkpoint therapy
title The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
title_full The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
title_fullStr The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
title_full_unstemmed The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
title_short The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
title_sort significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
topic Sarcomatoid
Rhabdoid
Dedifferentiation
Renal cell carcinoma
ISUP grade
Immune checkpoint therapy
url http://www.sciencedirect.com/science/article/pii/S2468294222001319
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