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

Pediatr Nephrol. 2010 Sep;25(9):1755-8. doi: 10.1007/s00467-010-1528-8. Epub 2010 Apr 23.


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.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Humans
  • Infant
  • Intestinal Polyps / complications
  • Kidney Neoplasms / complications
  • Kidney Neoplasms / diagnosis
  • Kidney Neoplasms / therapy*
  • Kidney Transplantation
  • Lung Neoplasms / complications
  • Male
  • Neoplasms, Cystic, Mucinous, and Serous / complications
  • Neoplasms, Cystic, Mucinous, and Serous / diagnosis
  • Neoplasms, Cystic, Mucinous, and Serous / therapy*
  • Neoplasms, Multiple Primary
  • Pleural Neoplasms / complications
  • Pulmonary Blastoma / complications
  • Renal Replacement Therapy*
  • Tomography, X-Ray Computed
  • Treatment Outcome