Localized cystic disease of the kidney

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Localized cystic disease of the kidney is a rare benign entity that should be separated from hereditary cystic kidney diseases and cystic renal tumours.[1]

It is also known as segmental cystic disease of the kidney, unilateral cystic disease of the kidney[1] and benign multilocular cyst of the kidney.[2]

General

  • Rare and benign.
  • No family history of polycystic kidney disease.

Presentation:

  • Usually an incidental finding (7 of 9 cases).
  • Hematuria - uncommon (2 of 9 cases).

Radiology:

Gross

Features:[1]

  • Focal cystic lesion(s) without a capsule involving the renal papillae +/-renal cortex.
    • Contain clear or light-yellow serous fluid.

Note:

  • Focal - only part of the kidney should be involved.

Microscopic

Features:[1]

  • Cystis with cuboidal or flat lining epithelium.
    • Contiguous with renal collecting ducts.
  • Renal parenchyma (renal tubules or glomeruli) with in (cyst) septations.
  • +/-Foamy histiocytes.
  • +/-Refractile material within the cysts.

Notes - negatives:

  • No significant inflammation.
  • No capsule.
  • No papillary hyperplasia.

DDx:

See also

References

  1. 1.0 1.1 1.2 1.3 Ding, Y.; Chen, L.; Deng, FM.; Melamed, J.; Fan, R.; Bonsib, S.; Zhou, M. (Apr 2013). "Localized cystic disease of the kidney: distinction from cystic neoplasms and hereditary polycystic diseases.". Am J Surg Pathol 37 (4): 506-13. doi:10.1097/PAS.0b013e318271eff9. PMID 23211292.
  2. 2.0 2.1 Bhat, S.; Thomas, A. (Oct 2007). "Benign multilocular cyst or multilocular cystic nephroma in adults: three case reports.". Indian J Surg 69 (5): 209-11. doi:10.1007/s12262-007-0025-1. PMID 23132986.