155 Elastin in pulmonary pathology demonstrated centrally, in what is now called AIS, an increase in the elastin content, contraction of the alveolar wall, and a consequent marked reduction in the amount of remaining alveolar air. This phenomenon of possible in-vivo collapse of peripheral lung tissue was recently given support in a radiological–pathological correlation, whereby the radiological solid appearance could only be explained by collapse of the lepidic parts of adenocarcinomas279. A gross picture of the resection specimen associated with in-vivo collapse is shown in Figure 2. Thus, collapse of the peripheral lung will not only occur ex vivo during surgery, but may also happen in vivo. Pathology of elastin The diseases in pulmonary pathology with a change in elastin configuration are summarized in Table 1. Table 1. Diseases in pulmonary pathology with a change in elastin configuration Elastin Disease Morphology Reference Loss Emphysema Irregular enlarged airspaces and reduction in peribronchiolar alveolar wall attachments Wright et al. 288 Kawabata et al.289 Langerhans cell histiocytosis Focal emphysematous change Fukuda et al.290 Granulomatous inflammation Initial reticulin fiber increase in and around granuloma, resulting in hyalinosis Mariani et al.291 Idiopathic interstitial pneumonias Fibrotic areas: focal elastolysis Honda et al.292 Increase Adenocarcinoma* Increased elastin in alveolar walls and/or the collapsed center Noguchi et al.10 Elastosis† Dense disorganized deposits of elastin Fukushima et al.274 Starcher et al.293 Apical cap Subpleural increase in alveolar walls Lagstein294 Pleuropulmonary fibroelastosis Subpleural and septal increase Tsubosaka et al.271 von der Th sen et al.295 Kinoshita et al.296 Idiopathic interstitial pneumonias Increased vascular elastin Parra et al.297 Organized infarct Collapsed entangled elastic fibers within a collagenous stroma Kawabata et al.298 Groshong et al.299 *Some, but not all, adenocarcinomas have increased amounts of elastin in alveolar walls and/or the collapsed center. †Elastosis is defined as ‘large aggregates of elastin fibers’/‘diffuse increase in elastin mass’, similarly as in other organs: breast, stomach, and papillary thyroid carcinoma300. The prognosis of patients with adenocarcinomas in the breast301 302 and lung274 is better in patients with elastosis than in those without elastosis. 12
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