Skip to main content

Other Cystic Kidney Diseases

  • Chapter
  • First Online:
Primer on Nephrology
  • 1995 Accesses

Abstract

Nephrologists are often asked to evaluate the significance of renal cysts, which are detected on diagnostic imaging unrelated to the kidney. In those over the age of 50 years, it is reported that up to 50% may have renal cysts on CT imaging, varying from the commonly seen simple cyst to rare hereditary cysts associated with renal dysfunction. These lesions are categorised according to the Bosniak classification, and advances in genetic sequencing mean that we are beginning to reveal the underlying genomic predisposition and biological mechanisms driving certain forms of cystic disease, which has implications for diagnostics and accurate disease classification. Simple cysts are benign, but for the more detrimental types of cysts, renal transplantation may be curative.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Rule AD, Sasiwimonphan K, Lieske JC, Keddis MT, Torres VE, Vrtiska TJ. Characteristics of renal cystic and solid lesions based on contrast-enhanced computed tomography of potential kidney donors. Am J Kidney Dis. 2012;59(5):611–8.

    Article  Google Scholar 

  2. Lee YJ, Kim MS, Cho S, Kim SR. Association between simple renal cysts and development of hypertension in healthy middle-aged men. J Hypertens. 2012;30(4):700–4.

    Article  CAS  Google Scholar 

  3. Eknoyan G. A clinical view of simple and complex renal cysts. J Am Soc Nephrol. 2009;20(9):1874–6.

    Article  Google Scholar 

  4. Terada N, Arai Y, Kinukawa N, Terai A. The 10-year natural history of simple renal cysts. Urology. 2008;71(1):7–11; discussion -2.

    Article  Google Scholar 

  5. Israel GM, Bosniak MA. An update of the Bosniak renal cyst classification system. Urology. 2005;66(3):484–8.

    Article  Google Scholar 

  6. Srivastava A, Kumar A, Agarwal A. Donors with renal cysts: a dilemma in decision making. Transplant Proc. 2003;35(1):30–1.

    Article  CAS  Google Scholar 

  7. Grantham JJ. Acquired cystic kidney disease. Kidney Int. 1991;40(1):143–52.

    Article  CAS  Google Scholar 

  8. Matson MA, Cohen EP. Acquired cystic kidney disease: occurrence, prevalence, and renal cancers. Medicine (Baltimore). 1990;69(4):217–26.

    Article  CAS  Google Scholar 

  9. Scandling JD. Acquired cystic kidney disease and renal cell cancer after transplantation: time to rethink screening? Clin J Am Soc Nephrol. 2007;2(4):621–2.

    Article  Google Scholar 

  10. Herrera GA. C-erb B-2 amplification in cystic renal disease. Kidney Int. 1991;40(3):509–13.

    Article  CAS  Google Scholar 

  11. Costa MZ, Bacchi CE, Franco M. Histogenesis of the acquired cystic kidney disease: an immunohistochemical study. Appl Immunohistochem Mol Morphol. 2006;14(3):348–52.

    Article  Google Scholar 

  12. Klatte T, Marberger M. Renal cell carcinoma of native kidneys in renal transplant patients. Curr Opin Urol. 2011;21(5):376–9.

    Article  Google Scholar 

  13. Truong LD, Krishnan B, Cao JT, Barrios R, Suki WN. Renal neoplasm in acquired cystic kidney disease. Am J Kidney Dis. 1995;26(1):1–12.

    Article  CAS  Google Scholar 

  14. Moch H, Cubilla AL, Humphrey PA, Reuter VE, Ulbright TM. The 2016 WHO classification of tumours of the urinary system and male genital organs-part A: renal, penile, and testicular tumours. Eur Urol. 2016;70(1):93–105.

    Article  Google Scholar 

  15. Fleming S. Renal cell carcinoma in acquired cystic kidney disease. Histopathology. 2010;56(3):395–400.

    Article  Google Scholar 

  16. Schwarz A, Vatandaslar S, Merkel S, Haller H. Renal cell carcinoma in transplant recipients with acquired cystic kidney disease. Clin J Am Soc Nephrol. 2007;2(4):750–6.

    Article  Google Scholar 

  17. Sarasin FP, Wong JB, Levey AS, Meyer KB. Screening for acquired cystic kidney disease: a decision analytic perspective. Kidney Int. 1995;48(1):207–19.

    Article  CAS  Google Scholar 

  18. Rahbari-Oskoui F, Mittal A, Mittal P, Chapman A. Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2014;9(2):406–15.

    Article  Google Scholar 

  19. Online Mendelian Inheritance in Man, OMIM® Johns Hopkins University (Baltimore, MD): McKusick-Nathans Institute of Genetic Medicine; [cited 2020 June 14]. Available from: https://omim.org.

  20. Devlin LA, Sayer JA. Renal ciliopathies. Curr Opin Genet Dev. 2019;56:49–60.

    Article  CAS  Google Scholar 

  21. Hildebrandt F, Omram H. New insights: nephronophthisis-medullary cystic kidney disease. Pediatr Nephrol. 2001;16(2):168–76.

    Article  CAS  Google Scholar 

  22. Eckardt KU, Alper SL, Antignac C, Bleyer AJ, Chauveau D, Dahan K, et al. Autosomal dominant tubulointerstitial kidney disease: diagnosis, classification, and management – A KDIGO consensus report. Kidney Int. 2015;88(4):676–83.

    Article  CAS  Google Scholar 

  23. Gokhale JA, Glenton PA, Khan SR. Characterization of Tamm-Horsfall protein in a rat nephrolithiasis model. J Urol. 2001;166(4):1492–7.

    Article  CAS  Google Scholar 

  24. Scolari F, Caridi G, Rampoldi L, Tardanico R, Izzi C, Pirulli D, et al. Uromodulin storage diseases: clinical aspects and mechanisms. Am J Kidney Dis. 2004;44(6):987–99.

    Article  CAS  Google Scholar 

  25. Devuyst O, Olinger E, Weber S, Eckardt KU, Kmoch S, Rampoldi L, et al. Autosomal dominant tubulointerstitial kidney disease. Nat Rev Dis Primers. 2019;5(1):60.

    Article  Google Scholar 

  26. Ward CJ, Hogan MC, Rossetti S, Walker D, Sneddon T, Wang X, et al. The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet. 2002;30(3):259–69.

    Article  Google Scholar 

  27. Lu H, Galeano MCR, Ott E, Kaeslin G, Kausalya PJ, Kramer C, et al. Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease. Nat Genet. 2017;49(7):1025–34.

    Article  CAS  Google Scholar 

  28. Shaikewitz ST, Chapman A. Autosomal recessive polycystic kidney disease: issues regarding the variability of clinical presentation. J Am Soc Nephrol. 1993;3(12):1858–62.

    Article  CAS  Google Scholar 

  29. Sweeney WE Jr, Avner ED. Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 2011;26(5):675–92.

    Article  Google Scholar 

  30. Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 2003;111(5 Pt 1):1072–80.

    Article  Google Scholar 

  31. Bergmann C, Guay-Woodford LM, Harris PC, Horie S, Peters DJM, Torres VE. Polycystic kidney disease. Nat Rev Dis Primers. 2018;4(1):50.

    Article  Google Scholar 

  32. Gunay-Aygun M, Font-Montgomery E, Lukose L, Tuchman Gerstein M, Piwnica-Worms K, Choyke P, et al. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013;144(1):112–21 e2.

    Article  Google Scholar 

  33. Buscher R, Buscher AK, Weber S, Mohr J, Hegen B, Vester U, et al. Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes. Pediatr Nephrol. 2014;29(10):1915–25.

    Article  Google Scholar 

  34. Fabris A, Anglani F, Lupo A, Gambaro G. Medullary sponge kidney: state of the art. Nephrol Dial Transplant. 2013;28(5):1111–9.

    Article  Google Scholar 

  35. Torregrossa R, Anglani F, Fabris A, Gozzini A, Tanini A, Del Prete D, et al. Identification of GDNF gene sequence variations in patients with medullary sponge kidney disease. Clin J Am Soc Nephrol. 2010;5(7):1205–10.

    Article  CAS  Google Scholar 

Patient Information and Guidelines

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Adam Rumjon .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Rumjon, A. (2022). Other Cystic Kidney Diseases. In: Harber, M. (eds) Primer on Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-76419-7_61

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-76419-7_61

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-76418-0

  • Online ISBN: 978-3-030-76419-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics