Congenital lung lesions comprise a broad spectrum of rare but clinically significant developmental abnormalities, including congenital cystic adenomatoid malformation, bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts, which are commonly surgically treated. Although the terms congenital cystic adenomatoid malformation, bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts are entrenched in clinical usage and comfortably correspond to rigid pathologic definitions, there is a considerable overlap in the findings. Disregarding the controversy about lesion nomenclature and classification, it is widely accepted that congenital lung lesions result from perturbations in lung and airway embryogenesis. It is generally accepted that both place (level in the tracheobronchial tree) and timing (gestational age) of the embryologic insult correlates with the type of lesion and histopathology that is manifested. The objective of this review is to briefly review normal lung development and to analyze the known molecular mechanisms underlying those diseases.

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Keywords Congenital cystic adenomatoid malformation, Congenital lobar emphysema, Congenital lung diseases, Congenital pulmonary airway malformation, Hox gene, Hox protein, Lung development, Pulmonary sequestration, Wnt protein, article, beta catenin, biological marker, biotransformation, cancer classification, cancer epidemiology, cell adhesion molecule, cell differentiation, cell interaction, cell proliferation, collagenase, cystic adenomatoid malformation, dickkopf 1 protein, endoderm, enzyme activation, enzyme activity, epithelium cell, fibroblast growth factor 1, fibroblast growth factor 10, fibroblast growth factor 2, fibroblast growth factor receptor, fibroblast growth factor receptor 4, forkhead transcription factor, gene activation, gene control, gene deletion, gene expression, gene mutation, gene targeting, gestational age, heart, histopathology, hox b5 protein, human, lung agenesis, lung alveolus epithelium, lung cyst, lung development, lung emphysema, lung lesion, lung sequestration, mesenchyme, molecular biology, morphogenesis, notochord, paracrine signaling, platelet derived growth factor A, platelet derived growth factor receptor, priority journal, protein domain, protein expression, protein interaction, proteoglycan, retinal dehydrogenase, retinal dehydrogenase 2, retinoic acid, retinoic acid receptor alpha, retinoic acid receptor beta, signal transduction, sonic hedgehog protein, thyroid transcription factor 1, tracheobronchial tree, tracheoesophageal fistula, transcription factor GATA 4, transcription factor GATA 6, transcription factor Sox2, transcription factor T bet, transcription factor TBX4, transforming growth factor beta, unclassified drug
Persistent URL dx.doi.org/10.1053/j.sempedsurg.2010.03.003, hdl.handle.net/1765/20208
Citation
Correia-Pinto, J, Gonzaga, S, Huang, Y, & Rottier, R. (2010). Congenital lung lesions-underlying molecular mechanisms. Seminars in Pediatric Surgery, 19(3), 171–179. doi:10.1053/j.sempedsurg.2010.03.003