Fibrosing mediastinitis

Abstract

Fibrosing mediastinitis is a rare benign disorder caused by proliferation of acellular collagen and fibrous tissue within the mediastinum. Although many cases are idiopathic, many (and perhaps most) cases in the United States are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young and present with signs and symptoms of obstruction or compression of the superior vena cava, pulmonary veins or arteries, central airways, or esophagus. There may be two types of fibrosing mediastinitis: focal and diffuse. The focal type usually manifests on computed tomographic (CT) or magnetic resonance (MR) images as a localized, calcified mass in the paratracheal or subcarinal regions of the mediastinum or in the pulmonary hila. The diffuse type manifests on CT or MR images as a diffusely infiltrating, often noncalcified mass that affects multiple mediastinal compartments. CT and MR imaging play a vital role in the diagnosis and management of fibrosing mediastinitis.