Mediastinum
Inflammatory disorders
Sclerosing mediastinitis

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 13 June 2018, last major update December 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Sclerosing mediastinitis[TI]

Cite this page: Gulwani, H. Sclerosing mediastinitis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/mediastinumsclerosingmediastinitis.html. Accessed July 21st, 2018.
Definition / 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
Diagrams / tables

Images hosted on other servers:

Etiology: table of causes

Table of clinical features

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

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

Microscopic (histologic) 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)
Microscopic (histologic) images

Images hosted on other servers:

Various images

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