Fibrosing Mediastinitis (Histoplasmosis-related)
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Imaging findings
An older radiograph shows fine linear opacities and volume loss in the right upper lobe. CT angiography reveals a soft tissue mass with dense calcification in the mediastinum and hilar region. This mass causes narrowing of the pulmonary artery and almost complete occlusion of the right superior pulmonary vein. There is also thickening of an adjacent airway and engorgement of bronchovascular bundles, suggestive of increased lymphatic and venous congestion in the affected distribution. Differences in lung attenuation indicate relative underperfusion, and airways are also compressed.
Key takeaways
Fibrosing mediastinitis, most commonly caused by Histoplasmosis in endemic areas, is a granulomatous inflammatory process characterized by dense mediastinal fibrosis and calcification. This process encases and compresses adjacent structures, typically affecting pulmonary veins first, followed by pulmonary arteries and then airways, leading to obstruction, volume loss, and compromised perfusion. There is currently no definitive effective medical treatment.
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