PMC3148482_01
Thoracic computed tomography. Bilateral and diffuse ground-glass attenuation opacities predominant in the lower lung lobes and thin-walled cystic lesions diffusely distributed in both lungs, as identified using thoracic computed tomography.
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Thoracic computed tomography demonstrated bilateral and diffuse ground-glass attenuation opacities, which were predominant in the lower lobes of the lungs, and thin-walled cysts (Figure 1).
Histological evaluation. Photomicrographs show pulmonary changes observed during the autopsy. A and B) Pulmonary tissue with LAM cysts (*) associated with alveolar hemorrhage (he). Note the smooth muscle tissue proliferation at the cyst wall (B, arrow). HMB-45 and alpha-smooth muscle actin-positive LAM cells at the cysts wall (arrows) are shown in C and D, respectively. E) HMB45-positive LAM cells within the small pulmonary artery wall (arrows). F) Disruption of the arterial elastic layer (*). The adjacent alveolar spaces indicate the presence of hemosiderin-laden macrophages (top right in E and F). A and B: H&E staining; C, D and E: immunohistochemistry; F: Verhoeff staining. Scale bars  =  50 microm.
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A histological examination during the autopsy revealed cystic lesions in the pulmonary tissue, with alveolar hemorrhaging and atypical smooth muscle cell proliferation at the cyst wall (Figures 2A and 2B). These cells were positive for antibodies against melanoma-associated antigen (HMB-45) and alpha-smooth muscle actin in the immunohistochemical analysis, which is consistent with LAM (Figures 2C and 2D). A disruption of the arterial elastic layer was also observed (Figures 2E and 2F). Hemosiderin-laden macrophages in the adjacent alveolar spaces were also identified (top right in Figures 2E and 2F).