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Kidney tumor - cysts, children, adult benign

Cystic non-neoplastic lesions

Acquired cystic kidney disease


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 18 September 2012, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
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● Three or more cysts per kidney in patients on long-standing hemo- or peritoneal dialysis for end stage renal disease (unrelated to underlying renal pathology)

Epidemiology
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● 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 long-term 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
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● May be due to uremia
● Cysts may form due to obstruction by oxalate crystals, fibrosis or hyperplasia

Clinical features
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● Increased (7-50x) risk of renal cell carcinoma (7% at 10 years), but death is rare (Clin Nephrol 2003;59:153)

Case reports
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● 36 year old man on CAPD for 6 years (Nephrol Dial Transplant 2002;17:500)

Gross description
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● Moderately enlarged kidneys (usually < 800 g) with cortical and medullary cysts containing clear fluid
● > 40% replacement of kidney with cysts

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

Renal transplant 12 years prior

With central scar

Micro description
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● 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

Micro images
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Various images


Fig 2-atypical papillary hyperplasia, fig 1 & 3-associated carcinomas


Fig c: non-tumorous stroma adjacent to cyst

Molecular description
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● 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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Autosomal dominant polycystic kidney disease: markedly enlarged kidneys up to 2-4 kg and with family history

Additional references
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eMedicine, J Clin Pathol 1977;30:868, Eur Radiol 2000;10:1716

End of Kidney tumor - cysts, children, adult benign > Cystic non-neoplastic lesions > Acquired cystic kidney disease


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