Diagnosis and Treatment of Pleuropulmonary Blastoma in Children: A Single-Center Report of 41 Cases

Retrospective analysis of 41 pediatric PPB cases reveals high misdiagnosis rate (51.2%) and variable presentation by pathologic type, with 5-year disease-free survival of 69.2%. Early recognition is critical in children with pneumothorax, pulmonary cysts, or DICER1-related findings to enable timely multidisciplinary treatment and prevent progression to more aggressive subtypes.