Kidney nontumor

Developmental & cystic diseases

Acquired cystic kidney disease



Topic Completed: 1 December 2011

Minor changes: 5 October 2021

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: acquired cystic kidney disease

Mandolin S. Ziadie, M.D.
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Cite this page: Ziadie MS. Acquired cystic kidney disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumoracquiredcyst.html. Accessed November 27th, 2021.
Definition / general
  • Three or more cysts per kidney in patients on longstanding hemo- or peritoneal dialysis for end stage renal disease (unrelated to underlying renal pathology)
Epidemiology
  • Occurs in 10% - 20% of patients within the first three years of dialysis, 50% within the first five years and 90% after ten years
  • Also occurs in patients with longterm uremia prior to dialysis
  • Males > females during first ten years of dialysis
  • Not restricted to adults; occurs in children and young adults on dialysis (Pediatr Nephrol 1997;11:447)
  • Frequency and severity not affected by online hemodiafiltration (Ren Fail 2009;31:555)
Pathophysiology
  • May be due to uremia
  • Cysts may form due to obstruction by oxalate crystals, fibrosis or hyperplasia
Clinical features
Case reports
Gross description
  • Moderately enlarged kidneys (usually < 800 g) with cortical and medullary cysts containing clear fluid
  • > 40% replacement of kidney with cysts
Gross images

Images hosted on other servers:

Left: acquired cystic disease; right: with renal cell carcinoma

Renal transplant 12 years prior

With central scar

Microscopic (histologic) description
  • Cysts lined by flattened or cuboidal epithelium that may show focal pseudopapillae with nuclear enlargement and loss of polarity
  • Cysts may contain oxalate crystals
  • Surrounding parenchyma shows global glomerulosclerosis, interstitial fibrosis and tubular atrophy
Molecular / cytogenetics description
  • Atypical epithelial proliferations associated with gains of #7, #12, #17, #20 and Y, suggesting they represent early neoplasms (Hum Pathol 2002;33:761)
Differential diagnosis
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