Pleuroparenchymal Fibroelastosis as a Late-Onset Pulmonary Toxicity after Treatment with Anticancer Chemotherapy for High-Risk Neuroblastoma

TL;DR

A rare lung disease called pleuroparenchymal fibroelastosis can develop years after chemotherapy for high-risk neuroblastoma in children.

Contribution

This case report highlights PPFE as a late-onset toxicity of anticancer chemotherapy in high-risk neuroblastoma patients.

Findings

PPFE occurred in a patient treated for high-risk neuroblastoma years after chemotherapy.

The condition is marked by pleural thickening, fibrosis, and poor survival outcomes.

Lung transplantation remains the only treatment option for this progressive disease.

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare, progressive, restrictive lung disease characterized by hypercarbic respiratory failure. In pediatrics, it has been described in patients with a history of malignancy who have received a bone marrow transplant, chemotherapy, or radiotherapy. It is characterized by pleural thickening, fibrosis, subpleural elastosis, and intraalveolar collagen deposits. Survival is poor, and the only therapy is lung transplantation. Here, we report a patient who developed PPFE as a late-onset pulmonary toxicity after treatment with anticancer chemotherapy for high-risk neuroblastoma (NB).

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