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Cystic Diseases of the Kidney Treatment & Management

  • Author: Thomas Patrick Frye, DO; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
 
Updated: Jan 28, 2015
 

Medical Care

Effective means of prevention or modulation of disease have not yet been identified. Current treatment is aimed at symptom control. In general, therapy is reserved for pain, hypertension, infection, renal salt wasting, and nephrolithiasis.

Inherited cystic renal disease

Autosomal dominant polycystic kidney disease

Patients with autosomal dominant polycystic kidney disease (ADPKD) have decreased ability to concentrate urine and should be encouraged to drink 1-2 L of water daily.

Generally, a blood pressure of 130/80 mm Hg is considered the treatment goal for hypertension in this population. Moderate hypertension may be treated with sodium restriction (ie, < 100 mEq/d), exercise, and weight control. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are effective in controlling hypertension in ADPKD. However, ACE inhibitors have been associated with reversible renal failure in polycystic kidney disease. Calcium channel blockers also are effective in managing hypertension in ADPKD.

Hypertension appears to correlate with the size of the cyst, and aspiration of renal cysts results in a reduction of blood pressure.[42]

Prevention of infection with appropriate precautions is important, particularly in women. Avoid urinary tract instrumentation whenever possible.

Treatment of infection involving cystic kidneys requires a prolonged course of antibiotics. Most cyst walls are permeable to polar antibiotics, including cephalosporins, penicillin derivatives, and aminoglycosides. Occasionally, cysts are relatively impermeable to these agents and require parenteral lipophilic antibiotics, such as ciprofloxacin, erythromycin, chloramphenicol, or a tetracycline. Clinical evaluation findings, including sterile urine, lack of fever, and no renal pain on deep palpation, should guide the route and duration of antibiotic therapy.

Autosomal recessive polycystic kidney disease

Infants with autosomal recessive polycystic kidney disease (ARPKD) should be delivered at a facility with a neonatal intensive care unit of level IV.[43] The newborn should be provided supportive therapy while the degree of pulmonary insufficiency and the etiology is reviewed.

Pulmonary hypoplasia is common and is responsible for 30-40% of mortality.[44] Pulmonary insufficiency can be treated with-high frequency ventilation. Pulmonary hypertension can be reversed with inhaled nitric oxide.

Dialysis may be required for renal failure. In one case series, neonates with ESRD before 28 days of age had 1-year survival of 52% and 5-year survival of 48% with peritoneal dialysis.[45]

With less severe childhood disease, edema often is a problem and is managed with sodium restriction and loop diuretics. Hypertension is controlled with salt restriction and antihypertensives, with particular emphasis on the use of ACE inhibitors and ARBs.

The ESCAPE trial (Endovascular Treatment for Small Core and Proximal Occlusion Ischemic Stroke) demonstrated that maintaining a mean arterial blood pressure below the 50th percentile for age, height, and sex in children with stage 2-4 chronic kidney disease can increase the length of time before patients progress to ESRD.[46] Currently, there are no specific guidelines for an ideal target blood pressure.[28]

Hypersplenism and associated thrombocytopenia should not be treated with splenectomy. Limitation from contact sports is recommended. Cholangitis secondary to hypersplenism should be treated with a prolonged course of intravenous antibiotics.

Juvenile nephronophthisis (JNPHP) and medullary cystic kidney disease

In patients with severe salt wasting, salt supplementation may improve renal function and slow renal demise. ESRD necessitates dialysis or renal transplantation.

Acquired cystic renal disease

In acquired renal cystic disease (ARCD), mild bleeding episodes may be managed with bed rest and analgesics.

In medullary sponge kidney (MSK), encourage patients with nephrolithiasis to produce 2 L of urine daily. Patients with hypercalciuria may benefit from oral thiazide diuretics. Patients may develop urinary tract infections and should be taught preventative measures.

In patients with simple cysts, a cyst infection usually requires a combination of antimicrobial and surgical management. Pathogens encountered most frequently in infected simple cysts include Enterobacteriaceae, staphylococci, and Proteus species.

Investigational therapy

Research has identified biochemical targets that may allow disease-modifying therapy for renal cystic disease, and several agents have been tested in randomized clinical trials. Results with mammalian target of rapamycin (mTOR) inhibitors were disappointing: In adults with ADPKD and early chronic kidney disease, 18 months of treatment with sirolimus did not halt polycystic kidney growth.[47] Studies of somatostatin analogues (octreotide, lanreotide, pasireotide) have yielded more encouraging results, and additional drugs are being tested.[48]

Vasopressin receptor activation results in increased levels of cyclic adenosine monophosphate (cAMP), and cAMP has been shown to be cystogenic. This provides the rationale for vasopressin receptor blockade. Tolvaptan is a vasopressin receptor antagonist with high affinity in humans. In a 3-year phase III clinical trial in patients with ADPKD, tolvaptan slowed the increase in total kidney volume and the decline in kidney function, compared with placebo, but was associated with a higher discontinuation rate, owing to adverse events.[49]

New candidate drugs are currently being investigated in the preclinical phase. Promising therapies include metformin, rosiglitazone, calcimimetics (R-568), and roscovitine, all of which have shown efficacy in animal models. Metformin and rosiglitazone both have mTOR-inhibiting effects but act on additional pathways. R-568 combats defective Ca2+ intracellular regulation, which favors proliferation, and has been shown to decrease renal fibrosis and late stage cystic volume but has no effect on overall cystic burden. Roscovitine is a cyclin-dependent kinase inhibitor that initiates cell cycle arrest and inhibits cystic disease in mouse models.[50, 51]

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Surgical Care

Surgical indications in renal cystic disease vary with the underlying disorder.

Multicystic dysplastic kidney (MCDK)

Previously, the involved kidney in patients with MCDK was routinely removed to prevent the subsequent development of symptoms. Currently, however, surgical excision is indicated only if the dysplastic kidney interferes with respiratory or digestive function or if significant hypertension has developed. Additionally, cyst rupture, which can occur spontaneously or secondary to trauma, may require emergent surgical intervention.

Inherited cystic renal disease

In autosomal dominant polycystic kidney disease (ADPKD), significant chronic pain may result from expansion of renal cysts. Needle aspiration is usually the first-line approach to symptomatic cysts.

Initial resolution and then return of symptoms with reaccumulation of cyst fluid increases the chance that a laparoscopic cyst decortication will eliminate the patient's pain.[52] However, for the management of severe pain, hypertension, hematuria, or infection, surgical excision may be preferred.

Complex cysts can be explored laparoscopically and treated appropriately based on intraoperative frozen sections. Laparoscopic techniques have been used with good results. Studies have reported good outcomes of laparoscopic cyst decortication using a retroperitoneal approach especially for posterior or lower pole lesions.[53, 54]

Percutaneous endocystolysis is another technique described for treatment of symptomatic cysts. The technique involves obtaining percutaneous access, dilating the tract, and then introducing a resectoscope with rollerball electrode to cauterize the internal surface of the cyst. A 13-year experience with this technique reported clinical improvement in 100% of the patients with minimal complications.[55]

Nephrectomy may be performed simultaneously with renal transplantation to create space for the transplanted kidney and to relieve symptoms associated with the native polycystic kidney. The timing of performing the nephrectomy in the transplant patient has been debated. Data suggest that open ipsilateral nephrectomy at the time of transplantation with staged contralateral native nephrectomy has fewer perioperative complications than performing a laparoscopic bilateral nephrectomy.[56] In extreme cases of liver enlargement, severe pain and wasting may result. Partial hepatectomy may alleviate these symptoms.

In autosomal recessive polycystic kidney disease patients with severe portal hypertension, sclerotherapy or portosystemic shunt placement may be necessary to control bleeding. Splenectomy may be indicated for splenomegaly with significant complications. Patients with ESRD can be treated successfully with kidney transplantation. In combination with peritoneal dialysis, kidney transplantation in one series was associated with a 5-year survival of 83%.[45]

In juvenile nephronophthisis (JNPHP) and medullary cystic kidney disease (MCKD), if transplantation is considered, selecting an older or unrelated donor is advisable to minimize the risk of the transplanted kidney also being affected with these diseases.

Acquired cystic renal disease

In acquired renal cystic disease, persistent or severe hemorrhage may necessitate nephrectomy or renal embolization. If a 3-cm renal mass suggestive of renal cell carcinoma (RCC) is noted, a partial or radical nephrectomy is indicated.

Simple renal cysts rarely require surgical management to relieve pain or obstruction. Treatment options include the following:

  • Aspiration
  • Sclerosis
  • Open resection
  • Endoscopic marsupialization and fulguration
  • Percutaneous resection
  • Laparoscopic resection

A randomized trial by Agarwal et al that compared percutaneous sclerotherapy versus laparoscopic unroofing for symptomatic renal cysts determined that both procedures were safe and had equal efficacy. These investigators also concluded that cyst aspiration with sclerotherapy was associated with lower morbidity and shorter hospital stay.[57]

Bosniak category III and IV renal cysts require surgical exploration. Approximately 50% of Bosniak category III cystic renal lesions are malignant. Management depends on the appearance of the lesion and varies from exploration and biopsy to nephrectomy. The current standard approach is open exploration with anticipated partial nephrectomy. However, as the experience with laparoscopic exploration and nephrectomy grows, this technique may prove equally reasonable.

Cystic clear cell renal cell carcinoma

Whether the patient has known pathologically diagnosed malignancy from biopsy or suspected malignancy based on Bosniak classification, a urologist can anticipate good surgical outcomes after resection. In a study of laparoscopic nephrectomy for cystic clear cell RCC, all patients treated were alive after 5 years and no patient had extrarenal disease at the time of surgery. These data suggest that patients with cystic RCC should expect to be cured after surgical resection, and furthermore should undergo nephron-sparing surgery when possible.[58]

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Contributor Information and Disclosures
Author

Thomas Patrick Frye, DO Clinical Fellow, Urologic Oncology Branch, National Cancer Institute, National Institutes of Health

Disclosure: Nothing to disclose.

Coauthor(s)

Steven Abboud Research Fellow, Medical Research Scholars Program, National Cancer Institute, National Institutes of Health

Steven Abboud is a member of the following medical societies: American Medical Association, American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Bradley Fields Schwartz, DO, FACS Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, Society of Laparoendoscopic Surgeons, Society of University Urologists, Association of Military Osteopathic Physicians and Surgeons, American Urological Association, Endourological Society

Disclosure: Nothing to disclose.

Additional Contributors

Edmund S Sabanegh, Jr, MD Chairman, Department of Urology, Glickman Urological and Kidney Institute, Cleveland Clinic Foundation

Edmund S Sabanegh, Jr, MD is a member of the following medical societies: American Medical Association, American Society of Andrology, Society of Reproductive Surgeons, Society for the Study of Male Reproduction, American Society for Reproductive Medicine, American Urological Association, SWOG

Disclosure: Nothing to disclose.

Acknowledgements

John M Corman, MD Clinical Associate Professor of Urology, University of Washington at Seattle; Consulting Staff, Department of Urology, Virginia Mason Medical Center

Disclosure: Nothing to disclose.

Alex Gorbonos, MD Assistant Professor, Department of Urology, Loyola University School of Medicine

Alex Gorbonos, MD is a member of the following medical societies: Alpha Omega Alpha, American Urological Association, Endourological Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Justin A Siegal, MD Radiologist, Department of Radiology, Virginia Mason Medical Center

Disclosure: Nothing to disclose.

Andrew T Trout, MD Resident Physician, Department of Radiology, University of Michigan Medical School

Andrew T Trout is a member of the following medical societies: American Medical Association, Phi Beta Kappa, Radiological Society of North America, and Sigma Xi

Disclosure: Nothing to disclose.

References
  1. Xu HW, Yu SQ, Mei CL, Li MH. Screening for intracranial aneurysm in 355 patients with autosomal-dominant polycystic kidney disease. Stroke. 2011 Jan. 42(1):204-6. [Medline].

  2. Bisceglia M, Galliani CA, Senger C, Stallone C, Sessa A. Renal cystic diseases: a review. Adv Anat Pathol. 2006 Jan. 13(1):26-56. [Medline].

  3. Kalyoussef E, Hwang J, Prasad V, Barone J. Segmental multicystic dysplastic kidney in children. Urology. 2006 Nov. 68(5):1121.e9-11. [Medline].

  4. Avner ED, Sweeney WE. Renal cystic disease: new insights for the clinician. Pediatr Clin North Am. 2006 Oct. 53(5):889-909, ix. [Medline].

  5. Wilson PD. Polycystic kidney disease. N Engl J Med. 2004 Jan 8. 350(2):151-64. [Medline].

  6. Gunay-Aygun M, Avner ED, Bacallao RL, Choyke PL, Flynn JT, Germino GG, et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference. J Pediatr. 2006 Aug. 149(2):159-64. [Medline].

  7. Gusmano R, Caridi G, Marini M, Perfumo F, Ghiggeri GM, Piaggio G, et al. Glomerulocystic kidney disease in a family. Nephrol Dial Transplant. 2002 May. 17(5):813-8. [Medline].

  8. Guay-Woodford LM. Renal cystic diseases: diverse phenotypes converge on the cilium/centrosome complex. Pediatr Nephrol. 2006 Oct. 21(10):1369-76. [Medline].

  9. Saunier S, Salomon R, Antignac C. Nephronophthisis. Curr Opin Genet Dev. 2005 Jun. 15(3):324-31. [Medline].

  10. Mostov KE. mTOR is out of control in polycystic kidney disease. Proc Natl Acad Sci U S A. 2006 Apr 4. 103(14):5247-8. [Medline].

  11. Shillingford JM, Murcia NS, Larson CH, Low SH, Hedgepeth R, Brown N, et al. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. PNAS. 2006 Apr 4. 103:5466-5471. [Medline].

  12. Baradhi KM, Abuelo GJ. Unilateral renal cystic disease. Kidney Int. 2012 Jan. 81(2):220. [Medline].

  13. Harris PC, Torres VE. Polycystic kidney disease. Annu Rev Med. 2009. 60:321-37. [Medline]. [Full Text].

  14. Thomsen HS, Levine E, Meilstrup JW, Van Slyke MA, Edgar KA, Barth JC, et al. Renal cystic diseases. Eur Radiol. 1997. 7(8):1267-75. [Medline].

  15. Choyke PL. Acquired cystic kidney disease. Eur Radiol. 2000. 10(11):1716-21. [Medline].

  16. Wetmore JB, Calvet JP, Yu AS, Lynch CF, Wang CJ, Kasiske BL, et al. Polycystic kidney disease and cancer after renal transplantation. J Am Soc Nephrol. 2014 Oct. 25(10):2335-41. [Medline]. [Full Text].

  17. Siqueira Rabelo EA, Oliveira EA, Silva JM, Oliveira DS, Colosimo EA. Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology. 2006 Nov. 68(5):1098-102. [Medline].

  18. Welch TR, Wacksman J. The changing approach to multicystic dysplastic kidney in children. J Pediatr. 2005 Jun. 146(6):723-5. [Medline].

  19. Grantham JJ, Torres VE, Chapman AB, Guay-Woodford LM, Bae KT, King BF, et al. Volume progression in polycystic kidney disease. N Engl J Med. 2006 May 18. 354(20):2122-30. [Medline].

  20. Cornec-Le Gall E, Audrézet MP, Chen JM, Hourmant M, Morin MP, Perrichot R, et al. Type of PKD1 mutation influences renal outcome in ADPKD. J Am Soc Nephrol. 2013 May. 24(6):1006-13. [Medline]. [Full Text].

  21. Hogan MC, Abebe K, Torres VE, Chapman AB, Bae KT, Tao C, et al. Liver Involvement in Early Autosomal-Dominant Polycystic Kidney Disease. Clin Gastroenterol Hepatol. 2014 Aug 9. [Medline].

  22. Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 2003 May. 111(5 Pt 1):1072-80. [Medline].

  23. Onal B, Kogan BA. Natural history of patients with multicystic dysplastic kidney-what followup is needed?. J Urol. 2006 Oct. 176(4 Pt 1):1607-11. [Medline].

  24. Torres, Vicente, Harris, Peter, Yves, Pirson. Autosomal dominant polycystic kidney disease. The Lancet. April 14, 2007. 369:1287-1301.

  25. Bergmann C. ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol. 2015 Jan. 30(1):15-30. [Medline]. [Full Text].

  26. Mercado-Deane MG, Beeson JE, John SD. US of renal insufficiency in neonates. Radiographics. 2002 Nov-Dec. 22(6):1429-38. [Medline].

  27. Bae KT, Zhu F, Chapman AB, Torres VE, Grantham JJ, Guay-Woodford LM, et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2006 Jan. 1(1):64-9. [Medline].

  28. [Guideline] Guay-Woodford LM, Bissler JJ, Braun MC, Bockenhauer D, Cadnapaphornchai MA, Dell KM, et al. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 2014 Sep. 165(3):611-7. [Medline].

  29. Chaumoitre K, Brun M, Cassart M, Maugey-Laulom B, Eurin D, Didier F, et al. Differential diagnosis of fetal hyperechogenic cystic kidneys unrelated to renal tract anomalies: A multicenter study. Ultrasound Obstet Gynecol. 2006 Dec. 28(7):911-7. [Medline].

  30. Hawkins JS, Dashe JS, Twickler DM. Magnetic resonance imaging diagnosis of severe fetal renal anomalies. Am J Obstet Gynecol. 2008 Mar. 198(3):328.e1-5. [Medline].

  31. Zaretsky M, Ramus R, McIntire D, Magee K, Twickler DM. MRI calculation of lung volumes to predict outcome in fetuses with genitourinary abnormalities. AJR Am J Roentgenol. 2005 Nov. 185(5):1328-34. [Medline].

  32. Borges Oliva MR, Hsing J, Rybicki FJ, Fennessy F, Mortele KJ, Ros PR. Glomerulocystic kidney disease: MRI findings. Abdominal Imaging. 2003 Nov. 28:889-892. [Medline].

  33. Song C., et al. Differential Diagnosis of Complex Cystic Renal Mass Using Multiphase Computerized Tomography. Journal of Urology. 2009/06. 181:2446-2450.

  34. Ellimoottil C, Greco KA, Hart S, Patel T, Sheikh MM, Turk TM, et al. New Modalities for Evaluation and Surveillance of Complex Renal Cysts. J Urol. 2014 Dec. 192(6):1604-1611. [Medline].

  35. Linguraru MG, Yao J, Gautam R, Peterson J, Li Z, Linehan WM, et al. Renal Tumor Quantification and Classification in Contrast-Enhanced Abdominal CT. Pattern Recognit. 2009 Jun 1. 42(6):1149-1161. [Medline]. [Full Text].

  36. Kim WB, Lee SW, Doo SW, Yang WJ, Song YS, Jeon JS, et al. Category migration of renal cystic masses with use of gadolinium-enhanced magnetic resonance imaging. Korean J Urol. 2012 Aug. 53(8):573-6. [Medline]. [Full Text].

  37. Bosniak MA. The use of the Bosniak classification system for renal cysts and cystic tumors. J Urol. 1997 May. 157(5):1852-3. [Medline].

  38. Garcia-Rojo, D. Comparison of Contrast-Enhanced Ultrasound (CE-US) and Computed Tomography (CT) in the Evaluation of Complex Cystic Renal Masses. Journal of Urology. May 31, 2010. 183:e243.

  39. Smith AD, Remer EM, Cox KL, et al. Bosniak category IIF and III cystic renal lesions: outcomes and associations. Radiology. 2012 Jan. 262(1):152-60. [Medline].

  40. Li, G.,, et al. CA9 molecular marker for differential diagnosis of cystic renal tumors. Urologic Oncology. Sept. 3, 2010.

  41. Torres VE, Harris PC. Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol. 2006 Jan. 2(1):40-55; quiz 55. [Medline].

  42. Zerem, E. Simple renal cysts and arterial hypertension: does their evacuation decrease the blood pressure?. Journal of Hypertension. Oct. 27, 2009. 10:2074-2078.

  43. [Guideline] Levels of neonatal care. Pediatrics. 2012 Sep. 130(3):587-97. [Medline].

  44. Guay-Woodford L. Other Cystic Diseases. Johnson R, Feehally J. Comprehensive Clinical Nephrology. 4th. London: Mosby; 2010. 543-559.

  45. Rheault MN, Rajpal J, Chavers B, Nevins TE. Outcomes of infants 11111111111Pediatr Nephrol</i>. 2009 Oct. 24(10):2035-9. [Medline].

  46. Wühl E, Trivelli A, Picca S, Litwin M, Peco-Antic A, Zurowska A, et al. Strict blood-pressure control and progression of renal failure in children. N Engl J Med. 2009 Oct 22. 361(17):1639-50. [Medline].

  47. Serra A., et al. Sirolimus and kidney growth in autosomal dominant polycystic kidney disease. New England Journal of Medicine. Aug. 26, 2010. 9:820-829.

  48. Wüthrich RP, Mei C. Pharmacological management of polycystic kidney disease. Expert Opin Pharmacother. 2014 Jun. 15(8):1085-95. [Medline].

  49. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, et al. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20. 367(25):2407-18. [Medline]. [Full Text].

  50. Chang MY, Ong AC. Mechanism-based therapeutics for autosomal dominant polycystic kidney disease: recent progress and future prospects. Nephron Clin Pract. 2012. 120(1):c25-34; discussion c35. [Medline].

  51. Aguiari G, Catizone L, Del Senno L. Multidrug therapy for polycystic kidney disease: a review and perspective. Am J Nephrol. 2013. 37(2):175-82. [Medline].

  52. Rane, A. Laparoscopic management of symptomatic simple renal cysts. International Urology Nephrology. 2004. 36:5-9.

  53. Emre, H. "Stepped procedure" in laparoscopic cyst decortications during the learning period of laparoscopic surgery: Detailed evaluation of initial experiences. Journal of Minimal Access Surgery. Apr 6, 2010. 2:37-41.

  54. Kilciler, M. Finger assisted laparoscopic renal cyst excision: a simple technique. Urology Journal. Jun 10, 2010. 7:90-94.

  55. Busato, W. Percutaneous endocystolysis, a safe and minimally invasive treatment for renal cysts: a 13-year experience. Journal of Endourology. Sept. 2010. 24:1405-1410.

  56. Lucas, S. Staged Nephrectomy Versus Bilateral Laparoscopic Nephrectomy in Patients with Autosomal Dominant Polycycstic Kidney Disease. Journal of Urology. Nov. 2010. 184:2054-2059.

  57. Agarwal M, Agrawal MS, Mittal R, Sachan V. A randomized study of aspiration and sclerotherapy versus laparoscopic deroofing in management of symptomatic simple renal cysts. J Endourol. 2012 May. 26(5):561-5. [Medline].

  58. Webster, W. Surgical Resection Provides Excellent Outcomes for Patients with Cystic Clear Cell Renal Cell Carcinoma. Urology. May 2007. 900-904.

  59. Bloom DA, Brosman S. The multicystic kidney. J Urol. 1978 Aug. 120(2):211-5. [Medline].

  60. Bonsib SM. Non-neoplastic diseases of the kidney. Bostwick DG. Urologic Surgical Pathology. St Louis, Mo: Mosby-Year Book; 1997.

  61. Bosniak MA. How does one deal with a renal cyst that appears to be Bosniak class II on a CT scan but that has sonographic features suggestive of malignancy (e.g., nodularity of wall or a nodular, irregular septum)?. AJR Am J Roentgenol. 1994 Jul. 163(1):216. [Medline].

  62. Bosniak MA. The current radiological approach to renal cysts. Radiology. 1986 Jan. 158(1):1-10. [Medline].

  63. Brook-Carter PT, Peral B, Ward CJ, Thompson P, Hughes J, Maheshwar MM, et al. Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease--a contiguous gene syndrome. Nat Genet. 1994 Dec. 8(4):328-32. [Medline].

  64. Chapman T. Fetal genitourinary imaging. Pediatr Radiol. 2012 Jan. 42 Suppl 1:S115-23. [Medline].

  65. Chauveau D, Duvic C, Chretien Y, Paraf F, Droz D, Melki P, et al. Renal involvement in von Hippel-Lindau disease. Kidney Int. 1996 Sep. 50(3):944-51. [Medline].

  66. Clarke A, Hancock E, Kingswood C, Osborne JP. End-stage renal failure in adults with the tuberous sclerosis complex. Nephrol Dial Transplant. 1999 Apr. 14(4):988-91. [Medline].

  67. Davidson AJ, Hartman DS, Choyke PL, Wagner BJ. Radiologic assessment of renal masses: implications for patient care. Radiology. 1997 Feb. 202(2):297-305. [Medline].

  68. Fick GM, Johnson AM, Hammond WS, Gabow PA. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995 Jun. 5(12):2048-56. [Medline].

  69. Freire M, Remer EM. Clinical and radiologic features of cystic renal masses. AJR Am J Roentgenol. 2009 May. 192(5):1367-72. [Medline].

  70. Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 2008 Oct 2. 359(14):1477-85. [Medline].

  71. Grantham JJ, Nair V, Winklhofer F. Cystic disease of the kidney. Brenner BM, ed. Brenner and Rector's the Kidney. 6th ed. Philadelphia, Pa: WB Saunders Co; 2000. 1171-1200.

  72. Harris PC, Bae KT, Rossetti S, Torres VE, Grantham JJ, Chapman AB, et al. Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2006 Nov. 17(11):3013-9. [Medline].

  73. Israel GM, Bosniak MA. Follow-up CT of moderately complex cystic lesions of the kidney (Bosniak category IIF). AJR Am J Roentgenol. 2003 Sep. 181(3):627-33. [Medline].

  74. Israel GM, Hindman N, Bosniak MA. Evaluation of cystic renal masses: comparison of CT and MR imaging by using the Bosniak classification system. Radiology. 2004 May. 231(2):365-71. [Medline].

  75. Lang EK, Macchia RJ, Gayle B, Richter F, Watson RA, Thomas R. CT-guided biopsy of indeterminate renal cystic masses (Bosniak 3 and 2F): accuracy and impact on clinical management. Eur Radiol. 2002 Oct. 12(10):2518-24. [Medline].

  76. Laube M, Hess B, Terrier F, Vock P, Jaeger P. [Prevalence of medullary sponge kidney in patients with and without nephrolithiasis]. Schweiz Rundsch Med Prax. 1995 Oct 24. 84(43):1224-30. [Medline].

  77. Levine E, Slusher SL, Grantham JJ, Wetzel LH. Natural history of acquired renal cystic disease in dialysis patients: a prospective longitudinal CT study. AJR Am J Roentgenol. 1991 Mar. 156(3):501-6. [Medline].

  78. Lonergan GJ, Rice RR, Suarez ES. Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. Radiographics. 2000 May-Jun. 20(3):837-55. [Medline].

  79. Matson MA, Cohen EP. Acquired cystic kidney disease: occurrence, prevalence, and renal cancers. Medicine (Baltimore). 1990 Jul. 69(4):217-26. [Medline].

  80. Miller MA, Brown JJ. Renal cysts and cystic neoplasms. Magn Reson Imaging Clin N Am. 1997 Feb. 5(1):49-66. [Medline].

  81. Ohlson L. Normal collecting ducts: visualization at urography. Radiology. 1989 Jan. 170(1 Pt 1):33-7. [Medline].

  82. Ravine D, Gibson RN, Walker RG, Sheffield LJ, Kincaid-Smith P, Danks DM. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994 Apr 2. 343(8901):824-7. [Medline].

  83. Siegel CL, McFarland EG, Brink JA, Fisher AJ, Humphrey P, Heiken JP. CT of cystic renal masses: analysis of diagnostic performance and interobserver variation. AJR Am J Roentgenol. 1997 Sep. 169(3):813-8. [Medline].

  84. Silverman SG, Israel GM, Herts BR, Richie JP. Management of the incidental renal mass. Radiology. 2008 Oct. 249(1):16-31. [Medline].

  85. Tsatsaris V, Gagnadoux MF, Aubry MC, Gubler MC, Dumez Y, Dommergues M. Prenatal diagnosis of bilateral isolated fetal hyperechogenic kidneys. Is it possible to predict long term outcome?. BJOG. 2002 Dec. 109(12):1388-93. [Medline].

  86. Wolf JS Jr. Evaluation and management of solid and cystic renal masses. J Urol. 1998 Apr. 159(4):1120-33. [Medline].

  87. Yent ER. Medullary sponge kidney. Schrier RE, Gottschalk CW. Disease of the Kidney. 5th ed. Little Brown: Boston, Mass; 1993. 525-32.

 
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Cut surface of a nephrectomy specimen from a patient with a multicystic dysplastic kidney (MCDK).
Nephrectomy specimen from a patient with a large benign simple cyst.
External surface of a nephrectomy specimen from a patient with autosomal dominant polycystic kidney disease (ADPKD).
Cut surface of the same nephrectomy specimen from a patient with autosomal dominant polycystic kidney disease (ADPKD).
Cut section of nephrectomy specimen demonstrating renal cell carcinoma (RCC), with an adjacent simple cyst.
Close-up photograph of the cut surface of the same nephrectomy specimen demonstrating a simple cyst adjacent to a renal cell carcinoma (RCC).
A prenatal sonogram of a fetus with a multicystic dysplastic kidney. The right kidney is appreciated as a large multicystic paraspinal mass. The left kidney and bladder are normal, and a normal amount of amniotic fluid is present.
CT examination of the abdomen of a 70-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) is shown. The kidneys are bilaterally enlarged with multiple cysts.
CT scan of the same patient (70-year-old woman with autosomal dominant polycystic kidney disease [ADPKD]) demonstrating multiple hepatic cysts.
This CT scan demonstrates acquired renal cystic disease (ARCD) in a 70-year-old man who is dialysis-dependent. The CT scan demonstrates bilateral atrophic kidneys with multiple renal cysts.
A CT scan of a 38-year-old man with von Hippel-Lindau syndrome (VHLS). The patient previously underwent resection of multiple bilateral renal cell carcinomas (RCCs). CT scan demonstrates multiple cysts in the kidneys and pancreas, as well as solid renal lesions suggestive of malignancy.
 
 
 
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