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Pathology for Urologists

Acquired Cystic Disease (ACD)


Image A
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Image B
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  • Development of multiple (>3) and bilateral renal cysts in patients whose CRF cannot be attributed to hereditary cystic disease.
  • >50% patients on dialysis for more than 4 years.
  • More common in males and African-American than Caucasian.
  • Clinical: Bilateral and asymptomatic in its early stages; cysts are present in 8% of patients at initiation of dialysis; 90% develop cysts after 10 years of dialysis.
  • Some cysts develop before dialysis, suggesting it's not only due to dialysis.
  • Mostly asymptomatic, some may present with abdominal pain and hematuria.
  • Gross:
    • Shrunken kidneys.
    • Cysts range from 0.5 - 3 cm in diameter.
    • Initially cortical in location, later medullary region too (image A).
  • Histology:
    • Tubular cysts in medulla and cortex.
    • Cysts are lined by flattened cuboidal or columnar epithelium (image B).
    • Generally involve the proximal tubules of the kidney.
    • May have calcium oxalate crystal depositions.
  • Occasional cysts contain clear cells that are carbonic anhydrase IX+; may be a precursor of RCC.
  • Should be accompanied by other changes of dialysis (end-stage) kidneys (sclerosis, fibrosis, atrophy, etc.).
  • Complications: RCC (50-100X increased risk), intrarenal and retroperitoneal hemorrhage and cyst infection.
  • 2-7% developed RCC, which include some unusual form (ACD-associated RCC and clear cell papillary RCC).
  • Large cysts require surgery to exclude RCC.

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