Mucinous adenocarcinoma of the lung in association with congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lung that has been associated with the presence of rhabdomyosarcoma, pleuropulmonary blastoma, and most commonly bronchioalveolar carcinoma (BAC) of the lung. Here, we report the case of an 8-year-old patient who developed KRAS mutation positive stage IV mucinous adenocarcinoma of the lung in association with CPAM. This case reflects the previously recognized progression of CPAM to malignancy and suggests that BAC arising in CPAM may take a more aggressive course than previously recognized.