Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations. Issue 11 (30th July 2020)

• Record Type: Journal Article
• Authors: Taylor, Bethany; Rice, Alexandra; Nicholson, Andrew G; Hind, Matthew; Dean, Charlotte H
• Is Part Of: Thorax. Volume 75:Issue 11(2020)
• Page Start: 1001
• Page End: 1003
• Publication Date: 2020-07-30
• Subjects: airway epithelium -- lung cancer -- TTF-1; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2020-214752
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract : 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.