Mediastinum
Nonneoplastic
Mediastinitis, sclerosing
Author: Hanni Gulwani, M.B.B.S.
Last author update: 1 December 2012
Last staff update: 15 March 2021
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Sclerosing mediastinitis[TI]
Table of Contents
Definition / general | Clinical features | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Differential diagnosisCite this page: Gulwani H. Mediastinitis, sclerosing. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mediastinumsclerosingmediastinitis.html. Accessed June 9th, 2023.
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
- 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
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)
Differential diagnosis
