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J Am Soc Nephrol. 2016 Aug;27(8):2227-37. doi: 10.1681/ASN.2015121335. Epub 2016 Mar 9.

The Nephrologist's Tumor: Basic Biology and Management of Renal Cell Carcinoma.

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Division of Kidney Disease and Hypertension, Warren Alpert Medical School of Brown University, Providence, Rhode Island;
Department of Pathology, University of Chicago, Chicago, Illinois;
Division of Nephrology, Yale University, New Haven, Connecticut; Medical Service Veterans Affairs Connecticut, West Haven, Connecticut;
Division of Nephrology and Center for Immunity, Inflammation and Regenerative Medicine, University of Virginia, Charlottesville, Virginia;
Renal Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Renal Division, Weill-Cornell Medical College, New York, New York;
Division of Nephrology and Cancer Center, University of California, Davis, California; and Medical Service, Veterans Affairs Northern California Health Care System, Sacramento, California


Kidney cancer, or renal cell carcinoma (RCC), is a disease of increasing incidence that is commonly seen in the general practice of nephrology. However, RCC is under-recognized by the nephrology community, such that its presence in curricula and research by this group is lacking. In the most common form of RCC, clear cell renal cell carcinoma (ccRCC), inactivation of the von Hippel-Lindau tumor suppressor is nearly universal; thus, the biology of ccRCC is characterized by activation of hypoxia-relevant pathways that lead to the associated paraneoplastic syndromes. Therefore, RCC is labeled the internist's tumor. In light of this characterization and multiple other metabolic abnormalities recently associated with ccRCC, it can now be viewed as a metabolic disease. In this review, we discuss the basic biology, pathology, and approaches for treatment of RCC. It is important to distinguish between kidney confinement and distant spread of RCC, because this difference affects diagnostic and therapeutic approaches and patient survival, and it is important to recognize the key interplay between RCC, RCC therapy, and CKD. Better understanding of all aspects of this disease will lead to optimal patient care and more recognition of an increasingly prevalent nephrologic disease, which we now appropriately label the nephrologist's tumor.


cancer; chronic renal disease; renal carcinoma

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