Pathology of Congenital Cystic Lung Diseases

Congenital pulmonary airway malformation (CPAM) type 1, CPAM type 2, bronchial atresia, and intralobar and extralobar pulmonary sequestration (IPS/EPS) are congenital cystic lung diseases (CCLD) that are most frequently submitted for pathological examination, whereas lobar emphysema, CPAM type 4, and bronchogenic cyst are less common. CPAM type 0, CPAM type 3, and alveolar capillary dysplasia are extremely rare. Pulmonary interstitial emphysema is not actually congenital, but usually occurs in the neonatal period, and a differential diagnosis with CCLD is often required. Fetal lung interstitial tumor (FLIT) is a tumorous lesion that usually occurs in the neonatal period, and sometimes in the fetal period.