Pediatric Nephrology

, Volume 1, Issue 3, pp 393–396 | Cite as

Human cystic kidney diseases: epithelial hyperplasia in the pathogenesis of cysts and tumors

  • Jay Bernstein
  • Andrew P. Evan
  • Kenneth D. GardnerJr.
International Symposium on Hereditary Nephropathies Heidelberg, 6–8 October, 1986


Several examples of human renal cystic disease are associated with tubular epithelial hyperplasia. Micropapillary hyperplasia occurs in autosomal dominant polycystic kidney disease, in localized cystic disease, and in acquired cystic disease; neoplastic or severely dysplastic epithelial hyperplasia occurs in von Hippel-Lindau disease; a histopathologically distinctive epithelial hyperplasia occurs in tuberous sclerosis. In all of these conditions the epithelial hyperplasia appears to be responsible for cyst formation by causing tubular or ductal luminal obstruction, and in all of these conditions, save localized cystic disease (a rare condition with very few reported cases), epithelial hyperplasia imposes an increased risk of malignancy. The risk seems to be highest in patients under treatment with long-term hemodialysis for end-stage kidney disease. Some of these diseases may share common features, but it appears likely that the histopathological differences reflect different features converging on a common result.

Key words

Polycystic kidney disease Renal carcinoma Tuberous sclerosis von Hippel-Lindau disease Acquired renal cystic disease 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Gardner KD Jr, Evan AP, Bernstein J (1978) Polyps in renal cystic disease. Lancet I: 878Google Scholar
  2. 2.
    Bernstein J (1979) Hereditary renal disease. In: Churg J, Abell MR (eds) The kidney. IAP Monograph No. 20. Williams and Wilkins, Baltimore, p 295Google Scholar
  3. 3.
    Evan AP, Gardner KD Jr (1976) Comparison of human polycystic and medullary cystic kidney disease with diphenylamine-induced cystic disease. Lab Invest 35: 93–101PubMedGoogle Scholar
  4. 4.
    Evan AP, Gardner KD Jr, Bernstein J (1979) Polypoid and papillary epithelial hyperplasia: a potential cause of ductal obstruction in adult polycystic disease. Kidney Int 16: 743–750PubMedGoogle Scholar
  5. 5.
    Bernstein J (1985) Morphology of human renal cystic disease. In: Cummings NB, Klahr S (eds) Chronic renal disease. Causes, complications and treatment. Plenum, New York, p 47Google Scholar
  6. 6.
    Huseman R, Grady A, Welling D, Grantham J (1980) Macropuncture study of polycystic disease in adult human kidneys. Kidney Int 18: 375–385PubMedGoogle Scholar
  7. 7.
    Grantham J (1986) Pathogenic mechanisms in cystic renal diseases. Invited lecture. Presented at the 18th Annual Meeting of the American Society of Nephrology, New Orleans, December 16Google Scholar
  8. 8.
    Ng RCK, Suki W (1980) Renal cell carcinoma occurring in polycystic kidney of a transplant recipient. J Urol 124: 710–712PubMedGoogle Scholar
  9. 9.
    Christoferson LA, Gustafson MB, Petersen AG (1961) von Hippel-Lindau's disease. JAMA 178: 126–128Google Scholar
  10. 10.
    Frimodt-Møller PC, Nissen HM, Dyreborg U (1981) Polycystic kidneys as the renal lesion in Lindau's disease. J Urol 126: 868–870Google Scholar
  11. 11.
    Christenson PJ, Craig JP, Bibro MC, O'Connell KJ (1982) Cysts containing renal cell carcinoma in von Hippel-Lindau disease. J Urol 128: 798–800PubMedGoogle Scholar
  12. 12.
    Bernstein J, Kissane JM (1973) Hereditary disorders of the kidney, part 1. Parenchymal defects and malformations. Perspect Pediatr Pathol 1: 117–146PubMedGoogle Scholar
  13. 13.
    Bernstein J, Robbins TO, Kissane JM (1986) The renal lesions of tuberous sclerosis. Semin Diagn Pathol 3: 97–105PubMedGoogle Scholar
  14. 14.
    Dunnill MS, Millard PR, Oliver D (1977) Acquired cystic disease of the kidneys: a hazard of long-term intermittent maintenance haemodialysis. J Clin Pathol 30: 868–877PubMedGoogle Scholar
  15. 15.
    Gardner KD Jr, Evan AP (1984) Cystic kidneys: an enigma evolves. Am J Kidney Dis 3: 403–413PubMedGoogle Scholar
  16. 16.
    Grantham J (1985) Acquired cystic disease: replacing one kidney disease with another. Kidney Int 28: 99–105PubMedGoogle Scholar
  17. 17.
    Gehrig JJ Jr, Gottheiner TI, Swenson RS (1985) Acquired cystic disease of the end-stage kidney. Am J Med 79: 609–619PubMedGoogle Scholar
  18. 18.
    Bretan PN Jr, Busch MP, Hricak H, Williams RD (1986) Chronic renal failure: a significant risk factor in the development of acquired, renal cysts and renal cell carcinoma. Case reports and review of the literature. Cancer 57: 1871–1879PubMedGoogle Scholar
  19. 19.
    Hughson MD, Buchwald D, Fox M (1986) Renal neoplasia and acquired cystic kidney disease in patients receiving long-term dialysis. Arch Pathol Lab Med 110: 592–601PubMedGoogle Scholar

Copyright information

© IPNA 1987

Authors and Affiliations

  • Jay Bernstein
    • 1
  • Andrew P. Evan
    • 2
  • Kenneth D. GardnerJr.
    • 3
  1. 1.Department of Anatomic PathologyWilliam Beaumont HospitalRoyal OakUSA
  2. 2.Department of AnatomyIndiana UniversityIndianapolisUSA
  3. 3.Department of MedicineThe University of New MexicoAlbuquerqueUSA

Personalised recommendations