Distal nephron neoplasms

Tumours of the distal nephron are uncommon but can create diagnostic difficulties. They may be divided into three groups—tumours of intercalated cell phenotype, those of principal cell phenotype and others with an unconfirmed distal nephron origin. Oncocytomas, chromophobe carcinoma and hybrid oncocytoma chromophobe carcinoma, all show features of intercalated cells and the distinction amongst these is one of the most common areas of diagnostic dilemma. Collecting duct carcinoma and renal medullary carcinoma are the most aggressive forms of renal cancer but recent evidence suggests they may respond to targeted therapy so their recognition becomes crucial to the management of these patients. There remains debate over the precise phenotype of both tubulocystic carcinoma and mucinous tubular and spindle cell carcinoma.