Predicting for Malignancy in Congenital Lung Malformations: A Single-Center Study of the "At Risk" Pediatric and Adult Population.
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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.