Congenital lung lesions--underlying molecular mechanisms.
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.
Correia-Pinto
Jorge
J
Surgical Sciences Research Domain, Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, Braga, Portugal. jcp@ecsaude.uminho.pt
Gonzaga
Sílvia
S
Huang
Yadi
Y
Rottier
Robbert
R
D001994:Bronchogenic Cyst / Q000151:congenital / Q000235:genetics / Q000628:therapy; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D002658:Developmental Disabilities / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D005260:Female; D047109:Fetal Development / Q000502:physiology; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities / Q000196:embryology*; D008297:Male; D008967:Molecular Biology; D011656:Pulmonary Emphysema / Q000151:congenital / Q000235:genetics / Q000628:therapy; D015619:Respiratory System Abnormalities / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D018570:Risk Assessment