Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.

The Genitourinary Pathology Society (GUPS) undertook a critical review of the recent advances in renal neoplasia, particularly focusing on the newly accumulated evidence post-2016 World Health Organization (WHO) classification. In the era of evolving histo-molecular classification of renal neoplasia, morphology is still key. However, entities (or groups of entities) are increasingly characterized by specific molecular features, often associated either with recognizable, specific morphologies or constellations of morphologies and corresponding immunohistochemical profiles. The correct diagnosis has clinical implications leading to better prognosis, potential clinical management with targeted therapies, may identify hereditary or syndromic associations, which may necessitate appropriate genetic testing. We hope that this undertaking will further facilitate the identification of these entities in practice. We also hope that this update will bring more clarity regarding the evolving classification of renal neoplasia and will further reduce the category of "unclassifiable renal carcinomas/tumors". We propose three categories of novel entities: (1) "Novel entity", validated by multiple independent studies; (2) "Emerging entity", good compelling data available from at least two or more independent studies, but additional validation is needed; and (3) "Provisional entity", limited data available from one or two studies, with more work required to validate them. For some entities initially described using different names, we propose new terminologies, to facilitate their recognition and to avoid further diagnostic dilemmas. Following these criteria, we propose as novel entities: eosinophilic solid and cystic renal cell carcinoma (ESC RCC), renal cell carcinoma with fibromyomatous stroma (RCC FMS) (formerly RCC with leiomyomatous or smooth muscle stroma), and anaplastic lymphoma kinase rearrangement-associated renal cell carcinoma (ALK-RCC). Emerging entities include: eosinophilic vacuolated tumor (EVT) and thyroid-like follicular renal cell carcinoma (TLFRCC). Finally, as provisional entities, we propose low-grade oncocytic tumor (LOT), atrophic kidney-like lesion (AKLL), and biphasic hyalinizing psammomatous renal cell carcinoma (BHP RCC).

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 2021 Feb 01 [Epub ahead of print]

Kiril Trpkov, Sean R Williamson, Anthony J Gill, Adebowale J Adeniran, Abbas Agaimy, Reza Alaghehbandan, Mahul B Amin, Pedram Argani, Ying-Bei Chen, Liang Cheng, Jonathan I Epstein, John C Cheville, Eva Comperat, Isabela Werneck da Cunha, Jennifer B Gordetsky, Sounak Gupta, Huiying He, Michelle S Hirsch, Peter A Humphrey, Payal Kapur, Fumiyoshi Kojima, Jose I Lopez, Fiona Maclean, Cristina Magi-Galluzzi, Jesse K McKenney, Rohit Mehra, Santosh Menon, George J Netto, Christopher G Przybycin, Priya Rao, Qiu Rao, Victor E Reuter, Rola M Saleeb, Rajal B Shah, Steven C Smith, Satish Tickoo, Maria S Tretiakova, Lawrence True, Virginie Verkarre, Sara E Wobker, Ming Zhou, Ondrej Hes

Department of Pathology and Laboratory Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada. ., Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA., Sydney Medical School, University of Sydney; Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research; NSW Health Pathology, Department of Anatomical Pathology, Royal North Shore Hospital, Sydney, NSW, Australia., Department of Pathology, Yale School of Medicine, New Haven, CT, USA., Institute of Pathology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany., Department of Pathology, Faculty of Medicine, University of British Columbia, Royal Columbian Hospital, Vancouver, BC, Canada., Department of Pathology and Laboratory Medicine and Urology, University of Tennessee Health Science, Memphis, TN, USA., Departments of Pathology and Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA., Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA., Departments of Pathology, Urology and Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA., Department of Pathology, Mayo Clinic, Rochester, MN, USA., Department of Pathology, Hôpital Tenon, Sorbonne University, Paris, France., Pathology Department, Rede D'OR-Sao Luiz, Sao Paulo, SP, Brazil., Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA., Department of Pathology, Health Science Center, Peking University, Beijing, China., Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA., Departments of Pathology, Urology, Kidney Cancer Program, Simmons Comprehensive Cancer Center, University of Texas Southwestern Medical Center, Dallas, TX, USA., Department of Human Pathology, Wakayama Medical University, Wakayama, Japan., Department of Pathology, Cruces University Hospital, Biocruces-Bizkaia Institute, Bizkaia, Spain., Department of Clinical Medicine, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, Australia., Department of Pathology, The University of Alabama at Birmingham, Birmingham, AL, USA., Department of Pathology and Michigan Center for Translational Pathology, University of Michigan Medical School, Ann Arbor, MI, USA., Department of Surgical Pathology, Tata Memorial Hospital, Parel, and Homi Bhabha National Institute, Mumbai, India., Department of Pathology, The University of Texas, MD Anderson Cancer Center, Houston, TX, USA., Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China., Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada., Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, TX, USA., Departments of Pathology and Urology, VCU School of Medicine, Richmond, VA, USA., Department of Pathology, University of Washington School of Medicine, Seattle, WA, USA., Department of Pathology, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris; Université de Paris, PARCC, INSERM, Equipe Labellisée par la Ligue contre le Cancer, F-75015, Paris, France., Departments of Pathology and Laboratory Medicine and Urology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA., Department of Pathology, Tufts Medical Center, Boston, MA, USA., Department of Pathology, Charles University in Prague, Faculty of Medicine and University Hospital in Plzen, Plzen, Czech Republic.