Pathology | Renal Cystic Diseases - [click on image(s) below]
Adult Polycystic Kidney Disease
Clinical: patients present in 30-50’s; usually complain of flank pain; 25% lack family history
Gross: Kidneys are massively enlarged and multicystic with essentially complete replacement of the renal cortex.
Hint: to distinguish this from acquired cystic disease (related to dialysis) evaluate the size of the kidneys: APKD will have large kidneys; kidneys with acquired cystic disease, on the other hand, will be small (because patients start off with shrunken kidneys of chronic renal failure before acquiring cystic changes)
Histology: cystic dilatation of terminal ends of ampullary collecting ducts, proximal convoluted tubules, and Bowman’s capsule
Syndrome: Autosomal dominant; this is the most common cystic renal disease and the most common genetically transmitted disease (1:500 - 1:1000)
Potter’s classification: Type III
Complications: metanephric abscesses; hypertension; berry aneurysms of the Circle of Willis; nephrolithiasis; and renal cell carcinoma (1-5% of cases)
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