Fibrosing Mediastinitis Secondary to Sarcoidosis
Imaging findings
Chest CT reveals a massive dilatation of the main pulmonary artery and an amorphous, non-calcified soft tissue mass within the mediastinum and hila. Progressive CT scans over one year demonstrate severe narrowing and near-complete occlusion of the bilateral inferior pulmonary veins due to extrinsic compression by the mediastinal fibrotic tissue, while biopsy of a mediastinal lymph node shows non-caseating granulomas.
Key takeaways
Fibrosing mediastinitis is characterized by the proliferation of invasive fibrous tissue within the mediastinum, most commonly secondary to histoplasmosis, but occasionally arising from sarcoidosis. The fibrotic process selectively compresses low-pressure vascular structures such as the pulmonary veins and superior vena cava, resulting in severe secondary pulmonary hypertension, and represents a distinct entity from histoplasmosis-related mediastinitis which is typically focal and densely calcified.
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