RT Journal Article
SR Electronic
T1 Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations
JF Thorax
JO Thorax
FD BMJ Publishing Group Ltd and British Thoracic Society
SP 1001
OP 1003
DO 10.1136/thoraxjnl-2020-214752
VO 75
IS 11
A1 Bethany Taylor
A1 Alexandra Rice
A1 Andrew G Nicholson
A1 Matthew Hind
A1 Charlotte H Dean
YR 2020
UL http://thorax.bmj.com/content/75/11/1001.abstract
AB Congenital pulmonary airway malformations (CPAMs) are rare lung abnormalities that result in cyst formation and are associated with respiratory distress in infants and malignant potential in adults. The pathogenesis of CPAMs remains unknown but data suggest disruption of the normal proximo-distal programme of airway branching and differentiation. Here, we demonstrate that adult human CPAM are lined with epithelium that retains SOX-2 and thyroid transcription factor-1 immunohistochemical markers, characteristic of the developing lung. However, RALDH-1, another key marker, is absent. This suggests a more complex aetiology for CPAM than complete focal arrest of lung development and may provide insight to the associated risk of malignancy.