Congenital lung disease: multiple entities you may not have encountered

Abstract Congenital lung disease comes to the attention of the surgical pathologist through lung biopsy or excision of a lung lobe. The two most frequent clinical indications are removal of a mass, often detected antenatally, or investigation of difficulty in ventilation or oxygenation. The diagnostic possibilities in each instance are different. The mass is most commonly a cystic airway malformation, but a limited number of rare neoplasms enters the differential diagnosis. Biopsy in the case of difficulty in ventilation most often shows delayed alveolar development, but surfactant protein disorder and a small number of lethal developmental abnormalities must be excluded. This review offers a conceptual framework to the general histopathologist confronted with such specimens and presents an approach to assessment in order to achieve the maximum information from the specimen. Correlation with clinical features and radiology is vital.