Acquired Cystic Disease



Acquired Cystic Disease


Anthony Chang, MD

Aleksandr Vasilyev, MD, PhD





Key Facts


Terminology



  • Presence of 3 or more cysts in native kidneys of patients with end-stage renal disease satisfies clinical diagnosis of ACD


Clinical Issues



  • 60-80% of patients on dialysis for more than 4 years may be diagnosed with ACD



    • Increased risk of developing renal cell carcinoma


    • 2-7% of patients with ACD will develop cystic renal cell carcinoma


  • Rapid change in cyst size warrants nephrectomy


  • Renal transplantation may decrease cyst size


Macroscopic Features



  • ACD kidney weights range from 5-800 grams with an average of 130 grams


  • Bilateral involvement


  • Masses or nodules that may represent carcinoma are common


Microscopic Pathology



  • Tubular cysts



    • Present throughout cortex and medulla


    • Lined by flat cuboidal epithelial cells


    • Most stain for proximal tubular markers


    • Intracystic hemorrhage may be present


  • Diffuse interstitial fibrosis and tubular atrophy


  • Calcium oxalate crystal deposition


Top Differential Diagnoses



  • Autosomal dominant polycystic kidney disease


  • Autosomal recessive polycystic kidney disease


  • Medullary cystic disease/juvenile nephronophthisis







Axial CECT shows innumerable cysts in bilaterally enlarged kidneys of a patient who has been on dialysis for many years.






Multiple cysts image are located throughout this end-stage kidney from a dialysis patient. This native kidney was removed for a suspicious mass image, which demonstrated both clear cell and papillary features.


TERMINOLOGY


Abbreviations



  • Acquired cystic disease (ACD)


Synonyms



  • Acquired cystic kidney disease


  • Acquired cystic renal disease


Definitions



  • Presence of 3 or more cysts in native kidneys of patients with end-stage renal disease satisfies clinical diagnosis of ACD


ETIOLOGY/PATHOGENESIS


Unknown Pathogenesis



  • Believed to involve physiologic response to chronic renal failure



    • Possible uncharacterized humoral factor



      • Successful renal transplantation may decrease size of cysts


    • Increased proliferation of cyst-lining epithelium


  • Unclear role of calcium oxalate crystals


  • Not necessarily secondary to dialysis



    • Cyst formation occurs in some patients even before dialysis therapy


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 60-80% of patients on dialysis for more than 4 years may be diagnosed with ACD


  • Age



    • All ages


  • Gender



    • Male predilection


  • Ethnicity



    • Africans may be more likely than Caucasians to develop ACD


Presentation

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Acquired Cystic Disease

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