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Pleuroparenchymal Fibroelastosis as a Late-Onset Pulmonary Toxicity after Treatment with Anticancer Chemotherapy for
Satoshi Yokoyama1, Risa Kanai1, Daisuke Fukao2
1Department of Pediatric Surgery, Japanese Red Cross Society, Wakayama Medical Center, 4-20 Komatsubara-dori, Wakayama 640-8558, Japan.
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).

