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. We describe a case of a fourteen-year-old female who presented with abdominal pain. A CT scan of the abdomen revealed a cystic lesion at the base of her right lung. Consequently, a CT arteriogram of the chest demonstrated a right lower lobe lesion concerning for type I CPAM. Following a thoracoscopic segmentectomy, histopathology revealed papillary adenocarcinoma with a KRAS mutation. Residual CPAM prompted a lobectomy, emphasizing the importance of surgical intervention for cystic lesions.