Sclerosing mediastinitis: a report on 18 cases.

Abstract

Conclusions -Sclerosing mediastinitis is a slowly progressive condition associated with previous tuberculosis, mediastinal malignancy, and autoimmune disease. The outlook is excellent for those cases without underlying malignancy. (Thorax 1995;50:280-283)

Mediastinal-Renal Syndrome: A Rare Manifestation of Granulomatosis with Polyangiitis

“…Fibrosing mediastinitis (FM) is a rare condition of obscure etiology characterized by a locally invasive fibroinflammatory process within the mediastinum [8,9]. FM is associated with substantial morbidity and mortality as a result of the compression and/or invasion of mediastinal blood vessels, heart, trachea, bronchial tree, and esophagus [8,9].…”

Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male

“…Other diseases that have been associated with fibrosing mediastinitis include autoimmune disease, Behçet disease, rheumatic fever and other idiopathic fibro-inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, Riedel thyroiditis, and pseudotumor of the orbit [6,7,10,11]. Trauma, radiation, methysergide maleate have also been associated with the development of fibrosing mediastinitis [5,6,12] Idiopathic cases are more often of the diffuse type while post inflammatory cases are more frequently localised [8].…”

“…Fibrosing mediastinitis is a rare condition, often progressive and potentially lethal, characterised by the proliferation of fibrous tissue and dense a-cellular collagen within the mediastinum occasionally containing patchy infiltrates of mononuclear cells or less commonly granulomas [6,7].…”

“…Such processes include infiltrative neoplastic disease and fibrosing mediastinitis [5,6]. Of the neoplastic diseases, a predominantly infiltrative growth pattern is seen in desmoplastic mesothelioma, metastatic carcinomas with fibrogenic inflammatory response, infiltrative thymomas, low grade sarcomas, sclerosing type of nonHodgkin lymphoma, and nodular sclerosis subtype of Hodgkin's disease [5].…”

“…A definite diagnosis and differentiation from fibrosing mediastinitis is obtained only by histology. Large tissue specimens and adequate sampling are essential to exclude malignancy associated with abundant desmoplastic reaction, making surgical biopsy necessary [6,7].…”