The disease concept of clear cell (tubulo) papillary renal cell carcinoma (CCP-RCC) as a distinct subtype of renal cell carcinoma has been recently established. First described in the setting of end stage renal disease, this tumor type is more frequently recognized and encountered in a sporadic setting. In this article, we provide an overview of the recent understanding of this tumor. Macroscopically, tumors are well circumscribed with well-developed tumor capsule. Histologically, the tumor cells are cuboidal to low columnar cell with clear cytoplasm and papillary and tubulo-papillary configuration. Immunohistochemically, tumor cells generally show diffuse expression for cytokeratin 7, CA9 (cup-shaped pattern), HIF-1, GLUT-1 and high molecular weight cytokeratin, but negative for AMACR, RCC Ma and TFE3. CD10 is negative or focally positive in most tumors. Genetically, this tumor has no characteristics of clear cell RCC or papillary RCC. Prognostically, patients with CCP-RCC behave in an indolent fashion in all previously reported cases. In conclusion, although this tumor has been integrated into recent International Society of Urologic Pathology Classification of renal neoplasia, both aspects of disease concept and clinical behavior are yet to be fully elucidated. Further publications of large cohorts of patients will truly help understand the biologic potential and the molecular underpinnings of this tumor type.

Department of Diagnostic Pathology Kobe University Hospital Kobe Japan

Department of Diagnostic Pathology Kochi Red Cross Hospital Kochi Japan

Department of Molecular Pathology Oita University Oita Japan

Department of Molecular Pathology Yokohama City University Graduate School of Medicine Yokohama Japan

Department of Pathology and Laboratory Medicine Cedars Sinai Medical Center Los Angeles CA USA

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

Department of Pathology Cruces University Hospital BioCruces Health Research Institute University of the Basque Country Barakaldo Bizkaia Spain

Department of Pathology Kansai Medical University Hirakata Hospital Osaka Japan

Department of Pathology National University Hospital System Singapore Singapore

Department of Pathology New York Medical Center NY USA

Division of Diagnostic Pathology Keio University Hospital Tokyo Japan

Division of Diagnostic Pathology Tokyo Women's Medical University Hospital Tokyo Japan

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