PMC4501529_01
a,b,c: Chest CT with mediastinal mass on right hilar region with right main bronchus caliber reduction, d: Right main bronchus caliber reduction and right pleural thickening.
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Hilar and subcarinal lymphadenopathy with calcifications was also present There was volume reduction of the right lung due to elevation of the diaphragm (Fig. 1).
Blunting of the Carina with extrinsic compression.
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Flexible bronchoscopy revealed marked inflammation and blunting of the carina, along with narrowing of both the main bronchi as well as trunchus intermedius (Fig. 2).
Pulmonary parenchyma with large vessels with thickened heterogenic walls and irregular form related to the hamartomatous nature of the disease. (a): Pulmonary parenchyma with large vessels. (b): Large vessels with thickened wall.
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Histological examination of the biopsy revealed - pulmonary parenchyma with large, irregular vessels with thickened heterogenic, irregular walls related to the hamartomatous nature of the disease (Fig. 3a-b) and pleural thickening with chronic inflammatory infiltrate and fibrosis.