case_images: PMC5700483_01
case_images: CT of chest showed area of emphysematous changes, several small lung cysts, patchy ground-glass opacity, interlobular septal thickening, and bronchiectasis.
case_images: CRIPU2017-9541419.001.jpg
case_images: PMC5700483_01_CRIPU2017-9541419.001.jpg
case_images: fig1
case_images: CT of chest performed at age 12 and age 21 are shown in Figures 1 and 2, respectively.
case_images: 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.
case_images: CRIPU2017-9541419.002.jpg
case_images: PMC5700483_01_CRIPU2017-9541419.002.jpg
case_images: fig2
case_images: CT of chest performed at age 12 and age 21 are shown in Figures 1 and 2, respectively.
case_images: (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.
case_images: CRIPU2017-9541419.003.jpg
case_images: PMC5700483_01_CRIPU2017-9541419.003.jpg
case_images: fig3
case_images: 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).
case_images: (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.
case_images: CRIPU2017-9541419.004.jpg
case_images: PMC5700483_01_CRIPU2017-9541419.004.jpg
case_images: fig4
case_images: They demonstrated a similar pattern of fibrosing NSIP, PNEC hyperplasia, and multiple carcinoid tumorlets, without presence of any granulomas (Figure 4).