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Epithelioid Hemangioendothelioma of the Pleura

Clinical and Radiologic Features

Eric J. Crotty1, H. Page McAdams1, Jeremy J. Erasmus1, Thomas A. Sporn2 and Victor L. Roggli2

1 Department of Radiology, Box 3808, Duke University Medical Center, Durham, NC 27710.
2 Department of Pathology, Duke University Medical Center, Durham, NC 27710.



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Fig. 1A. Epithelioid hemangioendothelioma of pleura in 66-year-old man with chest pain. Axial MR image (TR/TE, 606/20) shows infiltrating tumor (white arrows) in mediastinum that is encasing distal trachea (T). Note posteromedial pleural thickening (black arrows).

 


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Fig. 1B. Epithelioid hemangioendothelioma of pleura in 66-year-old man with chest pain. Sagittal MR image (550/20) shows tumor surrounding hilum (solid straight arrows) and extending into interlobar fissures (open straight arrows). Note small posterior loculated pleural effusion (e) and pleural thickening (curved arrow).

 


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Fig. 1C. Epithelioid hemangioendothelioma of pleura in 66-year-old man with chest pain. Photograph of histopathologic specimen of right lung taken at autopsy shows pseudomesotheliomatous tumor growth pattern. Note extension into hilum (long arrows) and along interlobar fissures (short arrows). H = hilum.

 


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Fig. 1D. Epithelioid hemangioendothelioma of pleura in 66-year-old man with chest pain. Photomicrograph shows positive immunoreactivity for factor VIII endothelial marker (brown-staining cells). Note intracytoplasmic lumina (arrows). (Immunoperoxidase stain with hematoxylin counterstain, x200)

 


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Fig. 1E. Epithelioid hemangioendothelioma of pleura in 66-year-old man with chest pain. Transmission electron photomicrograph shows ultrastructural features of endothelial differentiation: Weibel-Palade body (arrowhead), intermediate filaments, and pinocytotic vesicles (arrows).

 


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Fig. 2A. Epithelioid hemangioendothelioma of pleura in 55-year-old man with dyspnea and chest pain. Posteroanterior chest radiograph shows moderate right pleural effusion, pleural thickening, and nodularity (arrow).

 


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Fig. 2B. Epithelioid hemangioendothelioma of pleura in 55-year-old man with dyspnea and chest pain. Chest CT scan (mediastinal window setting) confirms loculated right effusion and pleural nodules (arrowheads).

 


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Fig. 2C. Epithelioid hemangioendothelioma of pleura in 55-year-old man with dyspnea and chest pain. Chest CT scan (lung window setting) shows scattered small pulmonary nodules (arrowheads). CT performed 6 months later (not shown) showed increased size and number of nodules, consistent with metastases.

 


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Fig. 3. Epithelioid hemangioendothelioma of pleura in 51-year-old man with dyspnea. Chest CT scan (mediastinal window setting) shows moderate right pleural effusion and pleural thickening. Note irregularity of subdiapragmatic fat (arrows), suggestive of diaphragmatic invasion that was confirmed at biopsy.

 


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Fig. 4A. Epithelioid hemangioendothelioma of pleura in 71-year-old man with severe dyspnea. Chest CT scans (mediastinal window setting) show loculated right pleural effusion and extensive pleural thickening (arrows, A). Note marked lymphadenopathy (n) in paratracheal, aortopulmonary window, and anterior paracardiac regions. Also note nodules along visceral and parietal pleura (arrowheads, B).

 


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Fig. 4B. Epithelioid hemangioendothelioma of pleura in 71-year-old man with severe dyspnea. Chest CT scans (mediastinal window setting) show loculated right pleural effusion and extensive pleural thickening (arrows, A). Note marked lymphadenopathy (n) in paratracheal, aortopulmonary window, and anterior paracardiac regions. Also note nodules along visceral and parietal pleura (arrowheads, B).

 

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