Papillary renal cell carcinoma represents approximately 10–15% renal neoplasms, and the name describes its characteristic papillary histology.  From a molecular standpoint, tumors are associated with trisomy 7, trisomy 17, and loss of chromosome Y (trisomy 7 in the familial form).  In contrast to clear cell renal cell carcinoma these tumors are NOT associated with 3p deletions.

 

The distal convoluted tubules of the kidney is thought to represent the cell of origin for this tumor.  The cells are typically cuboidal to slightly columnar and arranged in papillary formations.  Foamy histiocytes are common in the papillary cores.