PT  - JOURNAL ARTICLE
AU  - Bethany Taylor
AU  - Alexandra Rice
AU  - Andrew G Nicholson
AU  - Matthew Hind
AU  - Charlotte H Dean
TI  - Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations
AID  - 10.1136/thoraxjnl-2020-214752
DP  - 2020 Jul 29
TA  - Thorax
PG  - thoraxjnl-2020-214752
4099  - http://thorax.bmj.com/content/early/2020/07/29/thoraxjnl-2020-214752.short
4100  - http://thorax.bmj.com/content/early/2020/07/29/thoraxjnl-2020-214752.full
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.