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What is the treatment for idiopathic mediastinal fibrosis?

Q: I am providing the diagnostic result of my case; please advise regarding the treatment. Material: Mediastinal Biopsy. Clinical history provided: Idiopathic mediastinal fibrosis. Gross appearance: multiple grey brown fibrous pieces and grey brown soft and membranous tissue. Microscopic observations: Both sections studied show fibrous tissue with inflammatory cells to include plasma cells and lymphocytes. Mild phlebitis also noted. No evidence of malignancy in the material studied. Diagnosis: Features are consistent with the diagnosis of idiopathic mediastinal fibrosis.

A:This condition, if the histology is right, is very likely a post-inflammatory lesion called Sclerosing Mediastinitis and is best managed by proper surgery and steroids. I am copying below a short write up on the current information on the condition. It generally affects the anterior -superior mediastinum and often associated with vascular compromise symptoms. We do not know what the complaints are and surely the treating physician will advise correctly. Sclerosing mediastinitis - Also called idiopathic mediastinal fibrosis A fibroinflammatory lesion of mediastinum associated with other idiopathic fibrosing conditions such as retroperitoneal fibrosis, sclerosing cholangitis, Riedel’s struma and inflammatory pseudotumour of orbit. Also associated with pulmonary or mediastinal nodal infection due to Histoplasma or other fungi, trauma, syphilis, methysergide treatment and phlebitis. Usually anterosuperior mediastinum, presenting at any age, often with superior vena cava syndrome or cardiorespiratory compromise. X-ray: asymmetric mediastinal widening with projection of mass into upper lung field. Treatment: steroids, surgical excision Gross: dense white fibrosis, well demarcated from surrounding tissue Microscopy: different patterns - cellular fibrous reaction with polymorphic inflammatory infiltrate with plasma cells and eosinophils; dense, fibrohyaline tissue with focal spindle cells and inflammatory cells or scattered lymphoid follicles with occasional dystrophic calcification DD: fibrotic component of Hodgkin’s lymphoma, mesothelioma, metastatic carcinoma.


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