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The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia

Srigley, John R. MD*; Delahunt, Brett MD; Eble, John N. MD; Egevad, Lars MD, PhD§; Epstein, Jonathan I. MD; Grignon, David MD; Hes, Ondrej MD, PhD; Moch, Holger MD#; Montironi, Rodolfo MD**; Tickoo, Satish K. MD††; Zhou, Ming MD, PhD‡‡; Argani, Pedram MD§§The ISUP Renal Tumor Panel

The American Journal of Surgical Pathology: October 2013 - Volume 37 - Issue 10 - p 1469–1489
doi: 10.1097/PAS.0b013e318299f2d1
Special Articles

The classification working group of the International Society of Urological Pathology consensus conference on renal neoplasia was in charge of making recommendations regarding additions and changes to the current World Health Organization Classification of Renal Tumors (2004). Members of the group performed an exhaustive literature review, assessed the results of the preconference survey and participated in the consensus conference discussion and polling activities. On the basis of the above inputs, there was consensus that 5 entities should be recognized as new distinct epithelial tumors within the classification system: tubulocystic renal cell carcinoma (RCC), acquired cystic disease–associated RCC, clear cell (tubulo) papillary RCC, the MiT family translocation RCCs (in particular t(6;11) RCC), and hereditary leiomyomatosis RCC syndrome–associated RCC. In addition, there are 3 rare carcinomas that were considered as emerging or provisional new entities: thyroid-like follicular RCC; succinate dehydrogenase B deficiency–associated RCC; and ALK translocation RCC. Further reports of these entities are required to better understand the nature and behavior of these highly unusual tumors. There were a number of new concepts and suggested modifications to the existing World Health Organization 2004 categories. Within the clear cell RCC group, it was agreed upon that multicystic clear cell RCC is best considered as a neoplasm of low malignant potential. There was agreement that subtyping of papillary RCC is of value and that the oncocytic variant of papillary RCC should not be considered as a distinct entity. The hybrid oncocytic chromophobe tumor, which is an indolent tumor that occurs in 3 settings, namely Birt-Hogg-Dubé Syndrome, renal oncocytosis, and as a sporadic neoplasm, was placed, for the time being, within the chromophobe RCC category. Recent advances related to collecting duct carcinoma, renal medullary carcinoma, and mucinous spindle cell and tubular RCC were elucidated. Outside of the epithelial category, advances in our understanding of angiomyolipoma, including the epithelioid and epithelial cystic variants, were considered. In addition, the apparent relationship between cystic nephroma and mixed epithelial and stromal tumor was discussed, with the consensus that these tumors form a spectrum of neoplasia. Finally, it was thought that the synovial sarcoma should be removed from the mixed epithelial and mesenchymal category and placed within the sarcoma group. The new classification is to be referred to as the International Society of Urological Pathology Vancouver Classification of Renal Neoplasia.

*Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada

Department of Pathology and Molecular Medicine, Wellington School of Medicine, University of Otago, Wellington, New Zealand

Department of Pathology, Indiana University School of Medicine, Indianapolis, IN

Departments of Pathology, Urology and Oncology

§§Pathology and Oncology, Johns Hopkins Medical Institutions, Baltimore, MD

††Memorial Sloan Kettering Cancer Centre, NY

‡‡Department of Pathology, in New York University Medical Centre, New York, NY

§Department of Oncology and Pathology, Karolinska University Hospital Solna, Stockholm, Sweden

Department of Pathology, University Hospital Plzen, Plzen, Czech Republic

#Institute of Surgical Pathology, University of Zurich, Switzerland

**Section of Pathological Anatomy, Polytechnic University of Medicine, United Hospitals, Ancona, Italy

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: John R. Srigley, MD, Department of Laboratory Medicine, Trillium Health Partners, Credit Valley Hospital, 2200 Eglinton Avenue West, Mississauga, ON, Canada L5M 2N1 (e-mail:

© 2013 by Lippincott Williams & Wilkins.