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).

Anatomical Pathology Douglass Hanly Moir Pathology Sydney Australia

Department of Clinical Medicine Faculty of Medicine Health and Human Sciences Macquarie University Sydney Australia

Department of Human Pathology Wakayama Medical University Wakayama Japan

Department of Laboratory Medicine and Pathobiology University of Toronto Toronto ON Canada

Department of Pathology and Laboratory Medicine and Urology University of Tennessee Health Science Memphis TN USA

Department of Pathology and Laboratory Medicine Cumming School of Medicine University of Calgary Calgary AB Canada

Department of Pathology and Laboratory Medicine Indiana University School of Medicine Indianapolis IN USA

Department of Pathology and Michigan Center for Translational Pathology University of Michigan Medical School Ann Arbor MI USA

Department of Pathology Brigham and Women's Hospital Harvard Medical School Boston MA USA

Department of Pathology Charles University Prague Faculty of Medicine and University Hospital in Plzen Plzen Czech Republic

Department of Pathology Cruces University Hospital Biocruces Bizkaia Institute Bizkaia Spain

Department of Pathology Faculty of Medicine University of British Columbia Royal Columbian Hospital Vancouver BC Canada

Department of Pathology Health Science Center Peking University Beijing China

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

Department of Pathology Hôpital Tenon Sorbonne University Paris France

Department of Pathology Jinling Hospital Nanjing University School of Medicine Nanjing China

Department of Pathology Mayo Clinic Rochester MN USA

Department of Pathology Memorial Sloan Kettering Cancer Center New York NY USA

Department of Pathology Microbiology and Immunology Vanderbilt University Medical Center Nashville TN USA

Department of Pathology The University of Alabama at Birmingham Birmingham AL USA

Department of Pathology The University of Texas MD Anderson Cancer Center Houston TX USA

Department of Pathology The University of Texas Southwestern Medical Center Dallas TX USA

Department of Pathology Tufts Medical Center Boston MA USA

Department of Pathology University of Washington School of Medicine Seattle WA USA

Department of Pathology Yale School of Medicine New Haven CT USA

Department of Surgical Pathology Tata Memorial Hospital Parel and Homi Bhabha National Institute Mumbai India

Departments of Pathology and Laboratory Medicine and Urology University of North Carolina at Chapel Hill Chapel Hill NC USA

Departments of Pathology and Oncology The Johns Hopkins Medical Institutions Baltimore MD USA

Departments of Pathology and Urology VCU School of Medicine Richmond VA USA

Departments of Pathology Urology and Oncology The Johns Hopkins Medical Institutions Baltimore MD USA

Departments of Pathology Urology Kidney Cancer Program Simmons Comprehensive Cancer Center University of Texas Southwestern Medical Center Dallas TX USA

Institute of Pathology Friedrich Alexander University Erlangen Nürnberg University Hospital Erlangen Erlangen Germany

Pathology Department Rede D'OR Sao Luiz Sao Paulo SP Brazil

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

Erratum v

Mod Pathol. 2021 May;34(5):1037. doi: 10.1038/s41379-021-00775-0. PubMed

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