Congenital Pulmonary Airway Malformation Associated With Papillary Adenocarcinoma

Congenital pulmonary airway malformations (CPAMs) are cystic lung lesions often detected prenatally. Resection is often recommended for potential recurrent infections and malignancy. This report describes a case of a 14-year-old female patient who presented with abdominal pain. A computed tomographic scan of the abdomen revealed a cystic lesion at the base of her right lung. Consequently, a computed tomographic arteriogram of the chest demonstrated a right lower lobe lesion concerning for type I CPAM. After thoracoscopic segmentectomy, histopathologic examination revealed papillary adenocarcinoma with a KRAS mutation. Residual CPAM prompted a lobectomy, emphasizing the importance of surgical intervention for cystic lesions.