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.