Article Text

Download PDFPDF

Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations
  1. Bethany Taylor1,
  2. Alexandra Rice2,
  3. Andrew G Nicholson2,
  4. Matthew Hind1,3,
  5. Charlotte H Dean1
  1. 1 National Heart and Lung Institute, Imperial College London, London, UK
  2. 2 Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  3. 3 Respiratory Medicine, Department of Respiratory Medicine and National Institute for Health research Respiratory Biomedical Research Unit at the Royal Brompton NHS Foundation Trust and Imperial College, London, UK
  1. Correspondence to Dr Charlotte H Dean, National Heart and Lung Institute Division of Respiratory Science, London SW7 2AZ, UK; c.dean{at}


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
  • TTF-1

This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See:

Statistics from


  • 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.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.