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Kidney tumor - cysts, children, adult benign

Cystic non-neoplastic lesions

Glomerulocystic kidney


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 29 December 2011, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
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● Assorted group of disorders that share the common feature of glomerular cysts
● Classified based on association with other syndromes into four groups: familial GCKD, hereditary GCKD associated with ADPKD / ARPKD / tuberous sclerosis, syndromic but non-hereditary GCKD, sporadic and acquired GCKD
● Acquired includes associated with maternal phenacetin use during pregnancy (Arch Pathol Lab Med 1977;101:462), and hemolytic-uremic syndrome (Pediatr Nephrol 2001;16:557); syndromic includes hypomelanosis of Ito (Pediatr Nephrol 2008;23:1183)

Case reports
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● Newborns with tuberous sclerosis (Arch Pathol Lab Med 2000;124:327, Pathol Res Pract 1992;188:367)
● With tracheo-esophageal fistula and megacystic-megaureter syndrome (Diagn Pathol 2007 Apr 25;2:12)
● 55 year old woman (Nephrol Dial Transplant 2005;20:2293)

Gross description
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● Normal or enlarged kidney with cortical cysts

Gross images
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Bilateral spongy kidneys with microcysts in cortex

Micro description
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● Glomeruli with cystically dilated Bowmanís space and rudimentary or collapsed glomerular capillary tufts

Micro images
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Bilateral spongy kidneys with microcysts in cortex


Dilated Bowman's space with rudimentary glomerular capillary tufts


Bilateral cystic kidneys with megacystic-megaureter syndrome, kidney has occasional dysplastic tubules with immature mesenchymal collar (asterisk) and cystic glomeruli (arrows)


Glomeruli demonstrates marked dilation of Bowman's space and collapsed glomerular tufts


Newborn with tuberous sclerosis:
Fig 1: gross-sponge like kidney with numerous round, cystic spaces
Fig 2: low magnification EM-cystic spaces are variably sized with glomeruli somewhere along cyst wall (small arrows) and capsule (bottom right)
Fig 3: EM-cells covering Bowmanís capsule are tall and columnar (large arrow); hyperplasia is also present (curved arrows); glomerulus (fat arrow) is covered by plump, irregular podocytes
Fig 4: H&E shows Bowmanís capsule lining cells have eosinophilic granularity (big arrows)

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


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