Cyst development is found in both cortical and medullary regions. Most of the cysts are lined by non-descript, flattened to cuboidal epithelial cells. Some cysts however show epithelia with features of proximal tubules, distal tubules and collecting ducts. Tissues between the cysts may show unremarkable or atrophic nephron elements. The patients usually present in the fourth to fifth decade of life with progressive renal insufficiency and bilaterally enlarged cystic kidneys.
Hypertension is a common complication, perhaps on the basis of cysts causing local ischemia.
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