Ultrasound Imaging of Cystic Nephroma
Main Article Content
Keywords
cystic nephroma, cystic renal cell carcinoma, DICER1, mixed epithelial stromal tumor, renal cystic lesions
Abstract
Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term “cystic nephroma” represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections. In this review, we summarize the ultrasound imaging features of cystic nephroma and describe the characteristics of the most common renal cystic lesions and the differential diagnosis of cystic nephroma with other renal cystic lesions.
References
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22. Wilkinson C, Palit V, Bardapure M, Thomas J, Browning AJ, Gill K, et al. Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. Urol Ann. 2013 Jan;5(1):13–17. http://dx.doi.org/10.4103/0974-7796.106958
23. Kousari YM, Khanna G, Hill DA, Dehner LP. Case 211: Pleuropulmonary blastoma in association with cystic nephroma-DICER1 syndrome. Radiology. 2014 Nov;273(2):622–25. http://dx.doi.org/10.1148/radiol.14130949
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26. Dong B, Wang Y, Zhang J, Fu Y, Wang G. Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: A case report. J Can Urol Assoc. 2014 Jul;8(7–8):E545–7. http://dx.doi.org/10.5489/cuaj.2020
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28. Karmazyn B, Tawadros A, Delaney LR, Marine MB, Cain MP, Rink RC, et al. Ultrasound classification of solitary renal cysts in children. J Pediatr Urol. 2015 Jun;11(3):149.e1–6. http://dx.doi.org/10.1016/j.jpurol.2015.03.001
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36. Turbiner J, Amin MB, Humphrey PA, Srigley JR, Leval LD, Radhakrishnan A, et al. Cystic nephroma and mixed epithelial and stromal tumor of kidney: A detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (MEST) as a unifying term. Am J Surg Pathol. 2007 Apr;31(4):489–500. http://dx.doi.org/10.1097/PAS.0b013e31802bdd56
37. Grosse C, Sonnberger U, Grosse A, Breinl E, Hergan K. Multilocular cystic nephroma: Atypical sonographic and computed tomographic findings. Eur J Radiol Extra. 2006 Apr;58(1):13–16. http://dx.doi.org/10.1016/j.ejrex.2006.01.007
38. Lane BR, Campbell SC, Remer EM, Fergany AF, Williams SB, Novick AC, et al. Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney: Clinical, radiographic, and pathologic characteristics. Urology. 2008 Jun;71(6):1142–8. http://dx.doi.org/10.1016/j.urology.2007.11.106
39. Sahni VA, Mortele KJ, Glickman J, Silverman SG. Mixed epithelial and stromal tumour of the kidney: Imaging features. BJU Int. 2010 Apr;105(7):932–9. http://dx.doi.org/10.1111/j.1464-410X.2009.08918.x
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43. Kissane JM, Dehner LP. Renal tumors and tumor-like lesions in pediatric patients. Pediatr Nephrol. 1992 Jul; 6(4):365–82. http://dx.doi.org/10.1007/BF00869741
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2. Mohanty D, Jain BK, Agrawal V, Gupta A. Cystic nephroma: A diagnostic dilemma. Saudi J Kidney Dis Transpl. 2010 May;21(3):518–20.
3. Eble JN. Cystic nephroma and cystic partially differentiated nephroblastoma: Two entities or one? Adv Anat Pathol. 1994 Sep–Oct;1(2):99–102. http://dx.doi.org/10.1097/00125480-199409000-00007
4. Van Den Hoek J, De Krijger R, Van De Ven K, Lequin M, Van Den Heuvel-Eibrink MM. Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms’ tumor in children: A spectrum with therapeutic dilemmas. Urol Int. 2009 Jan;82(1):65–70. http://dx.doi.org/10.1159/000176028
5. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer. 1989 Jul;64(2):466–79. http://dx.doi.org/10.1002/1097-0142(19890715)64:2%3C466::AID-CNCR2820640221%3E3.0.CO;2-V
6. Michal M, Hes O, Bisceglia M, Simpson RH, Spagnolo DV, Parma A, et al. Mixed epithelial and stromal tumors of the kidney. A report of 22 cases. Virchows Arch. 2004 Oct;445(4):359–67. http://dx.doi.org/10.1007/s00428-004-1060-y
7. Jevremovic D, Lager DJ, Lewin M. Cystic nephroma (multilocular cyst) and mixed epithelial and stromal tumor of the kidney: A spectrum of the same entity? Ann Diagn Pathol. 2006 Apr;10(2):77–82. http://dx.doi.org/10.1016/j.anndiagpath.2005.07.011
8. Zhou M, Kort E, Hoekstra P, Westphal M, Magi-Galluzzi C, Sercia L, et al. Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney are the same disease entity: Molecular and histologic evidence. Am J Surg Pathol. 2009 Jan;33(1):72–80. http://dx.doi.org/10.1097/PAS.0b013e3181852105
9. Michal M, Amin MB, Delahunt B, Hes O, Oliva E. Mixed epithelial stromal tumor family. In: Moch H, Humphrey PA, Ulbright TM, Reuter VE, editors. World Health Organization classification of tumors: Pathology and genetics of tumors of the urinary system and male genital organs, 4th ed. Lyon: IARC Press; 2016. p. 70–1.
10. Argani P, Bruder E, Dehner L, Vujanic GM. Paediatric cystic nephroma. In: Moch H, Humphrey PA, Ulbright TM, Reuter VE, editors. World Health Organization classification of tumors: Pathology and genetics of tumors of the urinary system and male genital organs. 4th ed. Lyon: IARC Press; 2016. p. 53.
11. Bahubeshi A, Bal N, Rio Frio T, Hamel N, Pouchet C, Yilmaz A, et al. Germline DICER1 mutations and familial cystic nephroma. J Med Genet. 2010 Dec;47(12):863–6. http://dx.doi.org/10.1136/jmg.2010.081216
12. Slade I, Bacchelli C, Davies H, Murray A, Abbaszadeh F, Hanks S, et al. DICER1 syndrome: Clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet. 2011 Apr;48(4):273–8. http://dx.doi.org/10.1136/jmg.2010.083790
13. Mehraein Y, Schmid I, Eggert M, Kohlhase J, Steinlein OK. DICER1 syndrome can mimic different genetic tumor predispositions. Cancer Lett. 2016 Jan 28;370(2):275–8. http://dx.doi.org/10.1016/j.canlet.2015.11.002
14. Fernández-Martínez L, Villegas JA, Santamaría Í, Pitiot AS, Alvarado MG, Fernández S, et al. Identification of somatic and germ-line DICER1 mutations in pleuropulmonary blastoma, cystic nephroma and rhabdomyosarcoma tumors within a DICER1 syndrome pedigree. BMC Cancer. 2017 Feb 21;17(1):146. http://dx.doi.org/10.1186/s12885-017-3136-5
15. Murphy WM, Grignon DJ, Perlman EJ. Kidney tumors in children. In: Silverberg SG, Sobin LH, editors. Tumors of the kidney, bladder, and related urinary structures. Washington, DC: American Registry of Pathology; 2004. p. 1–99.
16. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: Radiologic-pathologic correlation. RadioGraphics. 1995 May;15(3):653–69. http://dx.doi.org/10.1148/radiographics.15.3.7624570
17. Vujanić GM. Renal tumours of childhood: An overview. Diagn Histopathol. 2009 Nov;15(11):501–9.
18. Li Y, Pawel BR, Hill DA, Epstein JI, Argani P. Pediatric cystic nephroma is morphologically, immunohistochemically, and genetically distinct from adult cystic nephroma. Am J Surg Pathol. 2017 Apr;41(4):472–81. http://dx.doi.org/10.1097/PAS.0000000000000816
19. Madewell JE, Goldman SM, Davis CJ Jr, Hartman DS, Feigin DS, Lichtenstein JE. Multilocular cystic nephroma: A radiographic-pathologic correlation of 58 patients. Radiology. 1983 Feb;146(2):309–21. http://dx.doi.org/10.1148/radiology.146.2.6294736
20. Bagley JE, Berry JL, McMurrian K. Case report: Multimodality imaging of a cystic nephroma. Radiol Technol. 2013 Jul–Aug;84(6):559–66.
21. Wani B, Kolte G, Rathod V, Banode P, Bhole A. Asymptomatic infantile cystic nephroma: A diagnostic dilemma. Indian J Surg. 2012 Dec;74(6):501–3. http://dx.doi.org/10.1007/s12262-011-0297-3
22. Wilkinson C, Palit V, Bardapure M, Thomas J, Browning AJ, Gill K, et al. Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. Urol Ann. 2013 Jan;5(1):13–17. http://dx.doi.org/10.4103/0974-7796.106958
23. Kousari YM, Khanna G, Hill DA, Dehner LP. Case 211: Pleuropulmonary blastoma in association with cystic nephroma-DICER1 syndrome. Radiology. 2014 Nov;273(2):622–25. http://dx.doi.org/10.1148/radiol.14130949
24. Aparanji G, Agarwal I, Chaturvedi S. Quiz page JULY 2014: Bilateral abdominal masses in an infant. Diagnosis: Bilateral cystic nephroma. Am J Kidney Dis. 2014 Jul;64(1):xvii–xix. http://dx.doi.org/10.1053/j.ajkd.2013.10.064
25. Pastor Navarro H, Martinez Sanchiz C, Peran Teruel M, Nam Cha SH, Pastor Guzman JM, Martinez Corcoles B, et al. Multilocular cystic nephroma: A clinical case. Arch Esp Urol. 2014 May;67(4):337–41.
26. Dong B, Wang Y, Zhang J, Fu Y, Wang G. Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: A case report. J Can Urol Assoc. 2014 Jul;8(7–8):E545–7. http://dx.doi.org/10.5489/cuaj.2020
27. Ozturk H, Karaaslan S. Uretheral invagination of multilocular cystic nephroma: A case report of a new pathologic variant. Int J Clin Exp Pathol. 2014 Jul 15;7(8):5271–9.
28. Karmazyn B, Tawadros A, Delaney LR, Marine MB, Cain MP, Rink RC, et al. Ultrasound classification of solitary renal cysts in children. J Pediatr Urol. 2015 Jun;11(3):149.e1–6. http://dx.doi.org/10.1016/j.jpurol.2015.03.001
29. Kurian JJ, Sen S, Joseph RT, Bindra MS. A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra. J Indian Assoc Pediatr Surg. 2015 Apr–Jun;20(2):82–3. http://dx.doi.org/10.4103/0971-9261.151553
30. Dell’Atti L. An unusual presentation of cystic nephroma in an adult man. Rare Tumors. 2015 May 18;7(2):5860. http://dx.doi.org/10.4081/rt.2015.5860
31. Mandal KC, Mukhopadhyay M, Barman S, Halder P, Mukhopadhyay B, Kumar R. Uncommon renal tumors in children: A single center experience. J Indian Assoc Pediatr Surg. 2016 Apr–Jun;21(2):61–5. http://dx.doi.org/10.4103/0971-9261.176940
32. Faure A, Atkinson J, Bouty A, O’Brien M, Levard G, Hutson J, et al. DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know. J Pediatr Urol. 2016 Feb;12(1):5–10. http://dx.doi.org/10.1016/j.jpurol.2015.08.012
33. Chung EM, Graeber AR, Conran RM. Renal tumors of childhood: Radiologic-pathologic correlation Part 1. The 1st decade: From the radiologic pathology archives. Radiographics. 2016 Mar–Apr;36(2):499–522. http://dx.doi.org/10.1148/rg.2016150230
34. Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol. 1951 Jun;23(2):142–52. http://dx.doi.org/10.1111/j.1464-410X.1951.tb02576.x
35. Boggs LK, Kimmelstiel P. Benign multilocular cystic nephroma: Report of two cases of so-called multilocular cyst of the kidney. J Urol. 1956 Nov;76(5):530–41. http://dx.doi.org/10.1016/S0022-5347(17)66732-6
36. Turbiner J, Amin MB, Humphrey PA, Srigley JR, Leval LD, Radhakrishnan A, et al. Cystic nephroma and mixed epithelial and stromal tumor of kidney: A detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (MEST) as a unifying term. Am J Surg Pathol. 2007 Apr;31(4):489–500. http://dx.doi.org/10.1097/PAS.0b013e31802bdd56
37. Grosse C, Sonnberger U, Grosse A, Breinl E, Hergan K. Multilocular cystic nephroma: Atypical sonographic and computed tomographic findings. Eur J Radiol Extra. 2006 Apr;58(1):13–16. http://dx.doi.org/10.1016/j.ejrex.2006.01.007
38. Lane BR, Campbell SC, Remer EM, Fergany AF, Williams SB, Novick AC, et al. Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney: Clinical, radiographic, and pathologic characteristics. Urology. 2008 Jun;71(6):1142–8. http://dx.doi.org/10.1016/j.urology.2007.11.106
39. Sahni VA, Mortele KJ, Glickman J, Silverman SG. Mixed epithelial and stromal tumour of the kidney: Imaging features. BJU Int. 2010 Apr;105(7):932–9. http://dx.doi.org/10.1111/j.1464-410X.2009.08918.x
40. White KS, Grossman H. Wilms’ and associated renal tumors of childhood. Pediatr Radiol. 1991;21(2):81–8. http://dx.doi.org/10.1007/BF02015609
41. Fishman EK, Hartman DS, Goldman SM, Siegelman SS. The CT appearance of Wilms tumor. J Comput Assist Tomogr. 1983 Aug;7(4):659–65. http://dx.doi.org/10.1097/00004728-198308000-00014
42. Reiman TA, Siegel MJ, Shackelford GD. Wilms tumor in children: Abdominal CT and US evaluation. Radiology. 1986 Aug;160(2):501–5. http://dx.doi.org/10.1148/radiology.160.2.3014599
43. Kissane JM, Dehner LP. Renal tumors and tumor-like lesions in pediatric patients. Pediatr Nephrol. 1992 Jul; 6(4):365–82. http://dx.doi.org/10.1007/BF00869741
44. Brisse HJ, Smets AM, Kaste SC, Owens CM. Imaging in unilateral Wilms tumour. Pediatr Radiol. 2008 Jan;38(1):18–29. http://dx.doi.org/10.1007/s00247-007-0677-9
45. Lee EY. CT imaging of mass-like renal lesions in children. Pediatr Radiol. 2007 Sep;37(9):896–907. http://dx.doi.org/10.1007/s00247-007-0548-4
46. Gylys-Morin V, Hoffer FA, Kozakewich H, Shamberger RC. Wilms tumor and nephroblastomatosis: Imaging characteristics at gadolinium-enhanced MR imaging. Radiology. 1993 Aug;188(2):517–21. http://dx.doi.org/10.1148/radiology.188.2.8392214
47. Murad T, Komaiko W, Oyasu R, Bauer K. Multilocular cystic renal cell carcinoma. Am J Clin Pathol. 1991 May;95(5):633–7. http://dx.doi.org/10.1093/ajcp/95.5.633
48. Corica FA, Iczkowski KA, Cheng L, Zincke H, Blute ML, Wendel A, et al. Cystic renal cell carcinoma is cured by resection: A study of 24 cases with long-term follow-up. J Urol. 1999 Feb;161(2):408–11. http://dx.doi.org/10.1016/S0022-5347(01)61903-7
49. Hartman DS, Davis CJ Jr, Johns T, Goldman SM. Cystic renal cell carcinoma. Urology. 1986 Aug;28(2):145–53. http://dx.doi.org/10.1016/0090-4295(86)90109-3
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Jul 20, 2017
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Ultrasound Imaging of Cystic Nephroma. (2017). Journal of Kidney Cancer, 4(3), 1-9. https://doi.org/10.15586/jkcvhl.2017.79
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Kidney Cancer: Review Articles
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How to Cite
Ultrasound Imaging of Cystic Nephroma. (2017). Journal of Kidney Cancer, 4(3), 1-9. https://doi.org/10.15586/jkcvhl.2017.79