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Acquired Cystic Disease-Associated Renal Cell Carcinoma

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  • Incidence of renal tumors is 100X in patents with end-stage kidney disease, particularly in ACKD.
  • Incidence of renal cancer in end stage kidney up to 7%.
  • Sporadic RCCs are common in ACKD, overall consisting 41% of tumors.
    • Clear cell RCC (18%)
    • Papillary RCC (15%)
    • Chromophobe RCC (8%)
  • Clear cell papillary RCC seen in 23%, was initially thought to be exclusive in ACKD but have also been described in non-ACKD (later).
  • ACKD-associated RCC constitutes 36%, and is exclusively seen ONLY in ACKD.
  • Shows gains of chromosomes 1, 2, 6 and 10.
  • Lacks +7 and +17, or 3p loss that characterizes papillary RCC and clear cell RR, respectively.

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  • Histology:
    • Characterized by tumor cell nests with multiple lumina imparting a sieve-like appearance
      (image A) & (image B) and abundance of calcium oxalate crystals (lights up with polarized light)
      (image C) & (image D).
    • Other architectures such as solid or papillary can be present.
  • Most are incidental tumor discovered radiologically on follow-up of patients with chronic renal disease.
  • Overall has favorable behavior, probably because most were detected at lower stage that can be attributed to the more regular medical care in patients with chronic renal disease.