ABSTRACT Introduction: Renal cell carcinoma (RCC) is generally divided into clear cell, papillary, and chromophobe subtypes. Clear cell papillary RCC is a recently described subtype. Case report: We report the case of a 42 year old female who was found on computed tomography scan of the abdomen to have a cyst which appeared to involve the right kidney. Pathology of the cyst wall revealed features consistent with a RCC, clear cell papillary type. Discussion: Clear cell papillary RCC are a recently described entity with less than 100 cases reported in the literature. Most cases in the literature have been associated with end-stage renal disease, however more case are being reported in patients without renal disease. Conclusion: Clear cell papillary RCC is a rare distinct entity in the spectrum of renal cell carcinoma. Based on the literature they appear to be clinically indolent tumors; however, more research is required to further characterize and prognosticate these rare tumors.
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