Predicting for Malignancy in Congenital Lung Malformations: A Single‐Center Study of the “At Risk” Pediatric and Adult Population

Introduction Congenital lung malformations (CLMs) are often identified on antenatal scanning and malignant transformation is cited as an indication for early resection. Long‐term follow‐up of asymptomatic lesions managed nonoperatively is lacking. Herein, we prospectively follow up children with antenatally identified CLMs and reviewed adult patients diagnosed with adenocarcinoma to look for background CLM changes. Methods This is a single‐center cohort study of pediatric patients with antenatal or early postnatal diagnosis of CLMs and a retrospective review of adult patients with lung adenocarcinoma. In children, data collected included type or location of CLMs and subsequent follow‐up. In adult patients, we reviewed radiology for background CLMs. Results One hundred and twenty‐nine pediatric patients diagnosed with CLMs: congenital pulmonary airway malformations ( n = 79; 61%), bronchopulmonary sequestrations ( n = 26; 20%), congenital lobar emphysema ( n = 11; 9%), resolved lesions ( n = 10; 8%), bronchogenic cysts ( n = 2; 2%), and an unknown lesion ( n = 1; 1%). The patients were followed up for 6.7 (IQR: 3.4–10.5; range 0.08–20.6) years. No pediatric patient was subsequently identified on the Northern Ireland Cancer Registry. Three hundred and twenty‐three adult patients were diagnosed with lung carcinoma: 37 (11%) were mucinous adenocarcinomas and 21 (57%) had a KRAS mutation. No CLMs were identified on review of radiological imaging in adult patients presenting with malignancy. Conclusion In children with CLMs, no resected malignant lesions could be identified on medium‐term follow‐up. Furthermore, no CLMs were identified in adult patients presenting with resected mucinous adenocarcinoma. Prospective collaborative databases are needed to determine true malignancy risk in asymptomatic CLM lesions and CPAMs.