Skip to main content
SpringerLink
Log in

Bilateral progressive cystic nephroma in a 9-month-old male infant requiring renal replacement therapy

  • Brief Report
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

We report the case of a 3-year-old boy who presented at 9 months of age with abdominal distension and was found to have a triad of bilateral cystic nephroma, pleuropulmonary blastoma (PPB) and juvenile intestinal polyps. There have been three previous reported cases of patients with the same associated diagnoses. Our patient is the first reported patient with PPB who received renal replacement therapy and progressed to successful renal transplantation. The potential increased risk of progression of malignancy of PPB (type 1) with immunosuppression following transplantation remains unknown.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Sacher P, Willi UV, Niggli F, Stallmach T (1998) Cystic nephroma: a rare benign renal tumour. Pediatr Surg Int 13:197–199

    Article  CAS  PubMed  Google Scholar 

  2. Polat P, Suma S, Celenk C, Alpay H, Tani V, Gundogdo C, Okur A (1997) Quiz case of the month. Multilocular cystic nephroma (MLCN). Eur Radiol 7:757–758

    Article  CAS  PubMed  Google Scholar 

  3. Sharma S, Nagar R, Singh K, Chrungoo RK (2000) Cystic nephroma: an unusual renal lesion. J Urol 163:1860

    Article  CAS  PubMed  Google Scholar 

  4. Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP (1988) Pleuropulmonary blastoma. The so-called Pleuropulmonary blastoma of childhood. Cancer 62:1516–1526

    Article  CAS  PubMed  Google Scholar 

  5. Boman F, Hill DA, Williams GM, Chauvenet A, Fournet JC, Soglio DB, Messinger Y, Priest JR (2006) Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumours: A report from the international pleuropulmonary blastoma registry. J Pediatr 149:850–854

    Article  PubMed  Google Scholar 

  6. Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohurt G, Freyer DR, Bostorm B, Richardson MS, Barredo J, Dehner LP (1996) Pleuropulmonary blastoma: a marker for familial disease. J Pediatr 128:220–224

    Article  CAS  PubMed  Google Scholar 

  7. Priest JR, Williams GM, Hill AD, Dehner LP, Jaffé A (2009) Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 44:14–30

    Article  PubMed  Google Scholar 

  8. Kural AR, Obeck C, Ozbay G, Onder AU (1998) Multilocular cystic nephroma: an unusual localisation. Urology 52:897–899

    Article  CAS  PubMed  Google Scholar 

  9. Ferrer FA, McKenna PH (1994) Partial nephrectomy in a metachronous mutlilocular cyst of the kidney (cystic nephroma). J Urol 151:1358–1360

    CAS  PubMed  Google Scholar 

  10. Lenz MP, Warmann SW, Scheel-Walter HG, Schäfer J, Wehrmann M, Hacker HW, Fuchs J (2005) A complicated case of bilateral cystic nephroma in a 16-month-old boy. Pediatr Surg Int 21:1011–1014

    Article  PubMed  Google Scholar 

  11. Sodhi KS, Suri S, Samujh R, Rao KL, Vaiphei K, Saxena AK (2005) Case report: bilateral multilocular cystic nephromas: a rare occurrence. Br J Radiol 78:450–452

    Article  CAS  PubMed  Google Scholar 

  12. Powell T, Shackman R, Johnson HD (1951) Multiocular cysts of the kidney. Br J Urol 23:142–152

    Article  CAS  PubMed  Google Scholar 

  13. Joshi VV, Beckwith JB (1989) Multilocular cyst of the kidney (cystic nephroma) and cystic partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer 64:466–479

    Article  CAS  PubMed  Google Scholar 

  14. Boulanger SC, Brisseau GF (2003) Cystic nephroma: A benign renal tumour of children and adults. Surgery 133:596–597

    Article  PubMed  Google Scholar 

  15. Dehner LP (1994) Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagn Pathol 11:144–151

    CAS  PubMed  Google Scholar 

  16. Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP (1997) Pleuropulmonary blastoma. A clinicopathologic study of 50 cases. Cancer 80:147–161

    Article  CAS  PubMed  Google Scholar 

  17. Delahunt B, Thomson KJ, Ferguson AF, Neale TJ, Meffan PJ, Nacey JN (1993) Familial cystic nephroma and pleuropulmonary blastoma. Cancer 71:1338–1342

    Article  CAS  PubMed  Google Scholar 

  18. Priest JR, Hill DA, Williams GM, Moertel CM, Messinger Y, Finkelstein MJ, Dehner LP (2006) Type I pleuropulmonary blastoma: A report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 24:4492–4498

    Article  PubMed  Google Scholar 

  19. Hill DA, Jarzembowski JA, Lennerz JK, Priest JR, Williams G, Schoettler P, Dehner LP (2008) Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry. Am J Surg Pathol 32:282–295

    Article  PubMed  Google Scholar 

  20. Hill DA, Ivanovich J, Priest JR, Gurnett CA, Dehner LP, Desruisseau D, Jarzembowski JA, Wikenheiser-Brokamp KA, Suarez BK, Whelan AJ, Williams G, Bracamontes D, Messinger Y, Goodfellow PJ (2009) DICER1 mutations in familial pleuropulmonary blastoma. Science 325:965

    Article  CAS  PubMed  Google Scholar 

  21. Bal N, Kayaselcuk F, Polat A, Bolat F, Yilmaz Z, Tuncer I (2005) Familial cystic nephroma in two siblings with pleuropulmonary blastoma. Pathol Oncol Res 11:53–56

    Article  PubMed  Google Scholar 

  22. Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H, Lapierre JG, Vischoff D, Tucci M, Brochu P (1999) Pleuropulmonary blastoma: a rare pathology with an even rarer presentation. J Pediatr Surg 34:1057–1059

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Paediatric Nephrology, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Ihab Sakr Shaheen, Maggie Fitzpatrick & Kay Tyerman

  2. Department of Paediatric Respiratory Medicine, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Keith Brownlee

  3. Department of Paediatric Radiology, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Nikhil Bhuskute

  4. Department of Paediatric Oncology, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Martin Elliott

  5. Department of Paediatric Surgery, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Mark Powis

  6. Department of Organ Transplantation, St. James’s University Hospital, Leeds, LS9 7TF, UK

    Niaz Ahmad

Authors
  1. Ihab Sakr Shaheen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Maggie Fitzpatrick
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Keith Brownlee
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Nikhil Bhuskute
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Martin Elliott
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Mark Powis
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Niaz Ahmad
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Kay Tyerman
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ihab Sakr Shaheen.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shaheen, I.S., Fitzpatrick, M., Brownlee, K. et al. Bilateral progressive cystic nephroma in a 9-month-old male infant requiring renal replacement therapy. Pediatr Nephrol 25, 1755–1758 (2010). https://doi.org/10.1007/s00467-010-1528-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-010-1528-8

Keywords

Search

Navigation