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Mediastinum

Inflammatory disorders

Sclerosing mediastinitis

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 February 2013, last major update December 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Also called idiopathic mediastinal fibrosis, fibrosing mediastinitis
● Fibroinflammatory lesion, usually anterosuperior mediastinum, often presenting with superior vena cava syndrome or cardiorespiratory compromise
● All ages

Etiology
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Table of causes

Clinical features
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● Associated with other idiopathic fibrosing conditions such as inflammatory pseudotumor of orbit, retroperitoneal fibrosis, Riedel’s struma, sclerosing cholangitis
● Also associated with pulmonary or mediastinal nodal infection due to fungi (Histoplasma), methysergide treatment, phlebitis, syphilis, trauma

Table of clinical features

Radiology
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● Asymmetric mediastinal widening with projection of mass into upper lung field
● Radiologically divided into 2 types: focal (common) and diffuse
  Focal: Localized and calcified mass in paratracheal or subcarinal compartments of mediastinum or in pulmonary hilum
  Diffuse: diffusely infiltrating, non-calcified mass affecting multiple mediastinal compartments
● Additional pulmonary findings includes infiltrates, consolidation and pleural effusion

Prognostic factors
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● Prognosis depends mainly on location of fibrosis and structures involved

Case reports
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● 30 year old man with idiopathic mediastinal fibrosis presenting as mediastinal compression syndrome (Indian J Med Sci 2005;59:268)
● 46 year old woman with tracheobronchial narrowing, severe hyperemia and mucosal edema (Rev Pneumol Clin 2009;65:159)
● 70 year old woman with multifocal fibrosclerosis with intracardiac solid masses (Hum Pathol 2006;37:493)
● Involving ascending aorta in setting of a multifocal fibrosclerotic disorder (Pathol Res Pract 2011;207:60)

Treatment
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● Steroids, surgical excision

Gross description
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● Tan-yellow, gelatinous masses to gray-white, hard fibrotic masses that compress or infiltrate mediastinal structures
● Often well demarcated from surrounding tissue

Gross images
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(a) Fibrosis encases the mediastinal vessels, thus causing stenosis

Micro description
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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
● Paucicellular areas with keloid like fibrosis may occur
● Necrosis and metaplastic bone can occasionally be present (Arch Pathol Lab Med 2010;134:417)

Micro images
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Various images


(b) Dense fibrosis with denser inflammation at the periphery
(c) Lamellar bands of fibrosis similar to a keloid scar

Differential diagnosis
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● Hodgkin lymphoma-nodular sclerosis
● Mesothelioma
● Metastatic carcinoma

End of Mediastinum > Inflammatory disorders > Sclerosing mediastinitis

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