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.
- airway epithelium
- lung cancer
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Contributors BT, CD and MH conceived and designed the study. BT, AR and CD conducted the study and acquired data. BT and CD prepared figures. BT, CD, MH and AN wrote the manuscript. All authors discussed the findings and reviewed and approved the manuscript.
Funding This work was funded by a Wellcome Trust Vacation Scholarship to BT, grant #WT20437/Z/16/Z and by The Royal Brompton and Harefield Hospitals charity through funding from Mr and Mrs Youssef Mansour, grant B1064.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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