Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Image Description

Renal Cell Carcinoma (RCC) in Autosomal Dominant Polycystic Kidney (ADPKD): ADPKD does not appear to increase the risk of RCC. The incidence of RCC in ADPKD is similar to that in the general population. However, when present, RCC in ADPKD is diagnosed at younger age, is more frequently bilateral and multicentric, and is more likely to have sarcomatoid morphology. The diagnosis of RCC in ADPKD is quite challenging, given the architectural distortion caused by cysts, hemorrhage, infection, and interstitial fibrosis.

This image from an ADPKD specimen shows variably-sized cysts separated from each other by thin septa. Some cysts contain pink proteinaceous material or hemorrhage. The cysts are lined by a single layer of flattened or low cuboidal epithelium.

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