Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

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
=========================================================================

● 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
=========================================================================



Table of causes

Clinical features
=========================================================================

● Associated with other idiopathic fibrosing conditions such as inflammatory pseudotumor of orbit, retroperitoneal fibrosis, Riedelís 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
=========================================================================

● 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
=========================================================================

● Prognosis depends mainly on location of fibrosis and structures involved

Case reports
=========================================================================

● 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
=========================================================================

● Steroids, surgical excision

Gross description
=========================================================================

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

Gross images
=========================================================================



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

Micro description
=========================================================================

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
=========================================================================



Various images


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


Differential diagnosis
=========================================================================

Hodgkin lymphoma-nodular sclerosis
Mesothelioma
Metastatic carcinoma

End of Mediastinum > Inflammatory disorders > Sclerosing mediastinitis


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).