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Unclassified renal cell carcinoma: diagnostic difficulties and treatment modalities

Authors Sirohi D, Smith SC, Agarwal N, Maughan BL

Received 20 June 2018

Accepted for publication 15 October 2018

Published 15 November 2018 Volume 2018:10 Pages 205—217

DOI https://doi.org/10.2147/RRU.S154932

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Amy Norman

Peer reviewer comments 4

Editor who approved publication: Dr Jan Colli


Deepika Sirohi,1 Steven C Smith,2,3 Neeraj Agarwal,4 Benjamin L Maughan4

1Department of Pathology, University of Utah and ARUP Laboratories, Salt Lake City, UT, USA; 2Department of Pathology, Virginia Commonwealth University, School of Medicine, Richmond, VA, USA; 3Department of Urology, Virginia Commonwealth University, School of Medicine, Richmond, VA, USA; 4Division of Medical Oncology, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA

Abstract: Over the past few decades, the classification system of renal cell carcinoma (RCC) variants has witnessed tremendous and ongoing refinement driven by genomic profiling and morphological correlation that have provided valuable insights into tumor biology and characterization of this heterogeneous subset of tumors. The importance of accurate classification cannot be understated given the downstream impact on treatment decisions, risk stratification, and need for genetic testing. While the morphologic heterogeneity across these tumors is increasingly being recognized, all non-clear-cell RCCs are commonly categorized under one therapeutic category with management strategies that largely derive from clear-cell RCCs. As research in metastatic RCC progresses, there is a growing focus on rare subtypes and unclassified tumors, which is rapidly changing the treatment paradigm for non-clear-cell RCC. This review focuses on the histomorphologic diagnostic challenges of unclassified RCCs discussing the utility of contemporary diagnostic tools. It further discusses the current state of knowledge and guidelines for management of this class of tumors.

Keywords: pathology, classification, morphology, management

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