Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma

Pediatr Pulmonol. 2020 Mar;55(3):E1-E4. doi: 10.1002/ppul.24638. Epub 2020 Jan 15.


Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.

Keywords: interstitial lung disease; pulmonary hypertension.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child, Preschool
  • Cough / etiology
  • Dyspnea / etiology
  • Female
  • Fibrosis
  • Humans
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / pathology
  • Neuroblastoma / diagnostic imaging
  • Neuroblastoma / drug therapy
  • Neuroblastoma / radiotherapy
  • Parenchymal Tissue / diagnostic imaging
  • Parenchymal Tissue / pathology*
  • Pleura / diagnostic imaging
  • Pleura / pathology*