Objective: The purpose of this article is to discuss the histopathologic features, genetics, clinical presentation, and imaging of hereditary renal cancer syndromes.
Conclusion: Hereditary renal cell carcinoma syndromes can be diagnosed with a pattern-based approach focused on the predominant histologic renal cell carcinoma subtype and associated renal and extrarenal features of each syndrome.
Keywords: chromophobe renal cell carcinoma; clear cell renal cell carcinoma; hereditary renal cell carcinoma syndromes; medullary renal cell carcinoma; papillary renal cell carcinoma; renal cell carcinoma.