Journal List > Tuberc Respir Dis > v.63(4) > 1001158

Shin, Kim, Park, Park, Park, Soon, Lee, Im, Jeon, Jung, Jeong, Choi, Kang, Choi, Hong, and Kim: Localized Fibrosing Mediastinitis with Superior Vena Caval(SVC) Syndrome

Abstract

Fibrosing mediastinitis is a rare disease that is characterized by the proliferation of dense fibrous tissue of the mediastinum. The pathogenesis of fibrosing mediastinitis is unknown in most cases. However, histoplasmosis, tuberculosis, autoimmune disease, radiation therapy, and other idiopathic fibroinflammatory diseases have been implicated in some cases. Most clinical features are related to an obstruction or compression of the mediastinal structure. Fibrosing mediastinitis is often progressive and occurs diffusely throughout the mediastinum. We encountered a case of fibrosing mediastinitis of a very focal lesion without evidence of mediastinal involvement. The condition was confirmed by biopsy and graft bypass surgery was performed because of SVC syndrome.

Figures and Tables

Figure 1
Engorged external jugular vein (A) and collateral vessels on the anterior chest wall and abdomen (B). After graft bypass, vein engorgements were improved (C).
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Figure 2
Initial chest PA shows cicatricial atelectasis and multiple cavities on left upper lung.
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Figure 3
In initial CT scans, about 3.5 × 2 cm sized ill defined infiltrative lesion was observed on SVC. SVC was totally obstructed by this lesion(arrow).
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Figure 4
Focal dense fibrous tissue compressed SVC at the junction of right innominate vein (4A, arrow). Within right inominate vein and right atrium, a 10 mm Gore-tex® graft used to bypass the obstruction site (4B).
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Figure 5
Dense collagenized fibrosis containing several chronic inflammatory cells and dark anthracotic pigmentation. Hematoxylin and Eosin stain. (5A: ×100, 5B: ×200).
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