PMC5573100_01
Posteroanterior (a) and lateral (b) chest X-rays. The chest radiography showed a diffuse nodular pulmonary pattern in both lungs.
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fig1
Chest X-ray demonstrated the presence of a diffuse and bilateral micronodular infiltrate (Figure 1).
Chest CT (a and b). Multislice chest CT-images on lung window showed innumerable, well-defined, small, round, and noncalcified pulmonary nodular opacities, scattered in both lungs. These nodules were present in all pulmonary lobes and adjacent to bronchioles and medium/small vessels.
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Chest high resolution (HR) computed tomography (CT) scan showed multiple, bilateral small pulmonary nodules (with diameters inferior to 10 mm) (Figure 2).
Contrast-enhanced multislice CT of the chest (a) on mediastinal window showed no thoracic lymphadenopathy. Multislice abdominopelvic CT scans (b and c) with intravenous contrast administration showed no significant findings, excluding focal liver or splenic lesions.
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Abdominal and pelvic CT revealed no malignant lesions (Figure 3).
Histopathologic findings of hemangioendothelioma. (a) Low magnification of the specimen obtained from pulmonary wedge resection reveals a subpleural nodule that extends to adjacent alveoli, with vascular proliferation in the peripheral zone and areas of recent and old hemorrhage. (b) Neoplastic nodule showing tumor cells with an eosinophilic stroma at the periphery. The lesion contains blood filled spaces and respiratory epithelium "trapped" within the lesion is also shown. (c) Higher magnification of the tumor reveals vacuolation of some of the tumor cells, representing primitive angiogenesis. One of these epithelioid cells can be observed with intracytoplasmic lumen and an erythrocyte inside, indicating their vascular nature. (d) Immunostaining for CD34 revealed positivity of the neoplastic cells (brown colour), confirming the endothelial lineage of the tumor.
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fig4
On immunohistochemical studies, the tumor cells were immunoreactive to CD34 and negative for cytokeratin (Figure 4).
PMC5573100_02
Posteroanterior (a) and lateral (b) chest X-rays. In a routine chest radiography 2 years before presentation in the ER it was difficult to detect the lesion in the left lower lung.
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In a routine chest radiograph made 2 years previously, it was hard to depict any pulmonary pathology (Figure 5).
Posteroanterior (a) and lateral (b) chest X-rays. The chest radiography showed a peripheral lesion in the left lower lung and a small volume of pleural effusion on the same side.
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Chest X-ray demonstrated the presence of a poorly defined opacity in the left lower lung and a blunt costophrenic angle in the left side (Figure 6).
Transbronchial biopsy of a pulmonary mass (H-E). (a) 10x. (b) 20x. Vascular proliferation and endothelial cells, frequently with citoplasmatic vacuolization, in a fibromyxoid stroma, with bronchial surface epithelial lining.
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The transbronchial biopsy showed atypical cellular infiltrate with immunohistochemical expression very suggestive of proliferative lesion of vascular origin (Figure 7).
Chest CT. Multislice chest CT-images on lung window (a, b, and c) showed a lung mass with irregular borders (a); they also showed multiple small, round, and noncalcified pulmonary nodular opacities, scattered in both lungs (b and c). On mediastinal window (d, e, and f) it is possible to better identify the pleural invasion (d) and the necrotic areas in lesion (e), retrospectively, and valorization is hard when the exam was performed, and we can recognize areas of enhancement in the left pleura, more nodular (arrow) or linear (arrowhead) (f).
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fig8
20 G Tru-Cut biopsy of the lung mass (a). H-E (b-e) images show vascular proliferation with epithelioid cells in a fibromyxoid stroma and presence of haemosiderophages ((b, c) 10x; (d) 20x). Some of the epithelioid cells show vacuolation (e). Immunostaining for CD31 revealed positivity of the neoplastic cells (brown colour), confirming the endothelial lineage of the tumor.
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fig9
She repeated the lung biopsy that showed the presence of atypical epithelioid-like tumor cells (Figure 9).
Thoracic MR images. Fluid sensitive sequences (a-c) show the presence of left pleural effusion (arrowhead) and also the nodular lesions in the spleen, which do not correspond to cysts (arrows). T1 weighted images with contrast (d and e) show the pulmonary mass and hypodense zones representing necrotic areas (arrow).
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Chest CT. Multislice chest axial CT-images on mediastinal window show (a) signs of previous atypical wedge resection in the left lower lobe (arrow); (b) more nodular lesions in the spleen (arrows); (c and d) more pronounced and diffuse pleural invasion (brackets).
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Also, showing increment in diameter, the splenic nodular lesions were compatible with metastases (Figure 11).
Chest CT. Multislice chest axial CT-images on mediastinal window (a, b, and c) show increase in size of the main lesion (a) and pleural metastases in the left side (arrows) and in contralateral side (arrowhead); a new lesion in the right lung is observed (arrow in (c)). Multislice chest axial CT-images on lung window (d and e) show the new contralateral lesion (arrow in (d)) and the parenchymal pulmonary nodules did not vary significantly in size from the previous studies (arrows in (e)).
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fig12
The pulmonary nodules, however, did not show significant increment in size despite the completely different evolution seen in the lesion of the lower left lung (Figure 12).
Multislice chest CT. Axial images on mediastinal window at the pulmonary bases (a and b) show increase in size and number of the nodular spleen lesions (arrows). A pleural effusion in the right side is also apparent (arrowhead).
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fig13
Although we did not have pathologic confirmation, because the patient did not have any other primary neoplastic lesion, these features reinforce the probable diagnosis of metastases from the PEH (Figure 13).