Mediastinum
Nonneoplastic
Mediastinitis, sclerosing


Topic Completed: 1 December 2012

Minor changes: 15 March 2021

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PubMed Search: Sclerosing mediastinitis[TI]

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Mediastinitis, sclerosing. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mediastinumsclerosingmediastinitis.html. Accessed May 6th, 2021.
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
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
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)
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