Clear Cell Papillary Renal Cell Carcinoma – A New Emerging Entity

Michael Sinnott, MD, Frank Chen, MD, PhD, Kazunori Kanehira, MD, Nobert Sule, MD, PhD, Bo Xu, MD, PhD


Clear cell papillary renal cell carcinoma was initially reported in patients with end stage renal disease under name of “end stage renal disease-associated renal cell carcinoma”. However, subsequent studies show that the tumor is also seen in non-end stage settings with or without impaired renal function. Recent advances in molecular genetic techniques and immunohistochemical staining have showed that clear cell papillary renal cell carcinoma is a new distinct entity that has unique genetic, histomorphological and clinical characteristics. This review summarizes the most current views on clear cell papillary renal cell carcinoma with focus on histomorphological features, immunohistochemical profiles and molecular genetic characteristic of this new entity.


clear cell papillary renal cell carcinoma, histomorphologic features, immunohistochemical and molecular-genetic characterization, pathoclinical correlation

Full Text:



Delahunt B, Eble JN. History of the development of the classification of renal cell neoplasia. Clin Lab Med. 2005;25(2):231-246.

Reuter VE, Presti JC, Jr. Contemporary approach to the classification of renal epithelial tumors. Semin Oncol. 2000;27(2):124-137.

Eble J, Sauter G, Epstein J, et al. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. Lyon: IARC Press; 2004.

Sule N, Yakupoglu U, Shen SS, et al. Calcium oxalate deposition in renal cell carcinoma associated with acquired cystic kidney disease: a comprehensive study. Am J Surg Pathol. 2005;29(4):443–451.

Tickoo SK, dePeralta-Venturina MN, Harik LR, et al. Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. Am J Surg Pathol. 2006;30(2):141–153.

Michal M, Hes O, Nemcova J, et al. Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity. Virchows Arch. 2009; 454(1):89–99.

Michal M, Hes O, Havlicek F. Benign renal angiomyoadenomatous tumor: a previously unreported renal tumor. Ann Diagn Pathol. 2000;4(5):311–315.

Aydin H, Chen L, Cheng L, et al. Clear cell tubulopapillary renal cell carcinoma: a study of 36 distinctive low-grade epithelial tumors of the kidney. Am J Surg Pathol. 2010;34(11):1608–1621.

Gobbo S, Eble JN, Grignon DJ, et al. Clear cell papillary renal cell carcinoma: a distinct histopathologic and molecular genetic entity. Am J Surg Pathol. 2008;32(8):1239–1245.

Behdad A, Monzon FA, Hes O, et al. Relationship between sporadic clear cell-papillary renal cell carcinoma (CP-RCC) and renal angiomyoadenomatous tumor (RAT) of the kidney: analysis by virtual-karyotyping, fluorescent in situ analysis and immunohistochemistry (IHC). Mod Pathol. 2011;24(Suppl):179A.

Herrera LP, Hes O, Hirsch M, et al. Clear cell-papillary renal cell carcinoma (CP-RCC) not associated with end stage renal disease: clinicopathologic analysis of 50 tumors confirming a novel subtype of renal cell carcinoma (RCC) occurring in a sporadic setting. Mod Pathol. 2011;24(Suppl):197A.

Fuhrman SA, Lasky LC, Limas C. Prognostic significance of morphologic parameters in renal cell carcinoma. Am J Surg Pathol. 1982;6(7):655–663.

Rohan SM, Xiao Y, Liang Y, et al. Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel–Lindau gene and hypoxia-inducible factor pathway-related proteins. Modern Pathol. 2011;24(9):1207–1220.

Mai KT, Faraji H, Desantis D, et al. Renal cell carcinoma with mixed features of papillary and clear cell cytomorphology: a fluorescent in situ hybridization study. Virchows Arch. 2010;456(1):77–84.

Argani P, Ladanyi M. Translocation carcinomas of the kidney. Clin Lab Med. 2005;25(2):363–378.

Argani P, Olgac S, Tickoo SK, et al. Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. Am J Surg Pathol. 2007;31(8):1149–1160.

Avery AK, Beckstead J, Renshaw AA, et al. Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms. Am J Surg Pathol. 2000;24(2):203–210.

Kim MK, Kim S. Immunohistochemical profile of common epithelial neoplasms arising in the kidney. Appl Immunohistochem Mol Morphol. 2002;10(4):332–338.

Tretiakova MS, Sahoo S, Takahashi M, et al. Expression of alpha-methylacyl-CoA racemase in papillary renal cell carcinoma. Am J Surg Pathol. 2004;28(1):69–76.

Delahunt B, Eble JN. Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors. Mod Pathol. 1997;10(6):537–544.

Martignoni G, Brunelli M, Gobbo S, et al. Role of molecular markers in diagnosis and prognosis of renal cell carcinoma. Anal Quant Cytol Histol. 2007;29(1):41–49.

Wolfe A, Dobin SM, Grossmann P, Michal M, Donner LR. Clonal trisomies 7, 10 and 12, normal 3p and absence of VHL gene mutation in a clear cell tubulopapillary carcinoma of the kidney. Virchows Arch. 2011;459(4):457-463.


  • There are currently no refbacks.