PMC5700483_01
CT of chest showed area of emphysematous changes, several small lung cysts, patchy ground-glass opacity, interlobular septal thickening, and bronchiectasis.
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fig1
CT of chest performed at age 12 and age 21 are shown in Figures 1 and 2, respectively.
CT of chest showed progressive fibrosis, with increased numbers of lung small cysts, as well as area of emphysema. Additional finding included subpleural honeycombing and mosaic attenuation.
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fig2
CT of chest performed at age 12 and age 21 are shown in Figures 1 and 2, respectively.
(a) Hematoxylin and eosin (H&E) stain 4x showed chronic interstitial pneumonia, overall pattern most consistently with NSIP. (b) H&E stain 10x showed alveolar septal thickening. (c) Synaptophysin 4x showed PNEC hyperplasia. (d) Synaptophysin 10x showed a carcinoid tumorlet sized approximately 2.5-3 mm.
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fig3
Histologic examination of the explanted lungs revealed fibrosing chronic interstitial pneumonia with an NSIP pattern and diffuse neuroendocrine cell hyperplasia with multiple carcinoid tumorlets (Figure 3).
(a) H&E stain 2x showed chronic interstitial pneumonia with alveolar septal thickening and fibrosis, pattern most suggestive of NSIP. (b) Synaptophysin stain 4x showed a carcinoid tumorlet. (c) Synaptophysin stain 40x showed PNEC hyperplasia.
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fig4
They demonstrated a similar pattern of fibrosing NSIP, PNEC hyperplasia, and multiple carcinoid tumorlets, without presence of any granulomas (Figure 4).