Pediatric Cystic Lung Lesions and Risk of Pleuropulmonary Blastoma: A Multi-Institutional Observational Cohort Study

Background: Pediatric cystic lung lesions represent a rare group of predominantly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. We aimed to examine the clinical presentation, pathology data, and diagnostic evaluation of children undergoing resection of a cystic lung lesion diagnosed after birth. Methods: A multisite observational cohort study was conducted of children undergoing resection of a postnatally diagnosed primary cystic lung lesion between Jan 1, 2009 and Dec 31, 2015 at one of 11 United States children’s hospitals (Midwest Pediatric Surgery Consortium). Clinical presentation, perioperative findings, and pathologic diagnosis were reviewed and analyzed using non-parametric bivariate and multivariable logistic regression models. Findings: Of 521 children with resected lung lesions, 177 (34·0%) underwent resection for a lesion that was not diagnosed prenatally. Operations was performed with low morbidity at median ages of 4.4 months [interquartile range (IQR), 0·9-28·7], 10·9 months (IQR, 7·6-151·2), and 42·3 months (IQR, 11·1-86·2) for respiratory symptoms (n=89, 50·6%), asymptomatic disease (n=35, 19·9%), and pneumonia history (n=54, 30.7%), respectively. The most common pathologic diagnosis was congenital pulmonary airway malformation (n=59, 34·3%). Fifteen (8·5%) were classified as malignant tumors [type 1 pleuropulmonary blastoma (PPB), n=11; other PPB, n=3; adenocarcinoma, n=1) at a median resection age of 20.7 months (IQR, 7·9-58·1). The sensitivity of preoperative chest CT for detecting malignant pathology was 33.3% (95% CI: 15·2-58·3). Multivariable logistic regression showed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology [odds ratios, 42·15 (95% CI: 7·43-340·3, p