Kidney nontumor
Developmental & cystic diseases
Glomerulocystic kidney

Topic Completed: 1 December 2011

Minor changes: 1 October 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Glomerulocystic kidney [title]

Mandolin S. Ziadie, M.D.
Page views in 2020: 665
Page views in 2021 to date: 271
Cite this page: Ziadie MS. Glomerulocystic kidney. website. Accessed May 15th, 2021.
Definition / general
  • 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 nonhereditary 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
Gross description
  • Normal or enlarged kidney with cortical cysts
Gross images

Images hosted on other servers:

Bilateral spongy kidneys with microcysts in cortex

Microscopic (histologic) description
  • Glomeruli with cystically dilated Bowman’s space and rudimentary or collapsed glomerular capillary tufts
Microscopic (histologic) images

Images hosted on other servers:

Bilateral spongy kidneys with microcysts in cortex

Dilated Bowman's
space with rudimen-
tary glomerular
capillary tufts

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

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 Bowman’s capsule; tall and columnar (large arrow); hyperplasia is also present (curved arrows); glomerulus (fat arrow) covered by plump, irregular podocytes
Fig 4: H&E shows Bowman’s capsule lining cells have eosinophilic granularity (big arrows)
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