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Mediastinum

Other benign / low grade tumors

Fibromatosis

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 1 March 2013, last major update December 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Anterior or posterior mediastinum

Epidemiology
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● Children and young adults

Clinical features
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● Associated with superior vena cava syndrome, nerve entrapment or dysphagia

Case reports
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● 48 year old man with intra-thoracic desmoid tumor and with a previous aortocoronary bypass (World J Surg Oncol 2006;4:43)
● 61 year old man with de novo fibromatosis and no history of thoracotomy or trauma (Rev Mal Respir 2008;25:82)

Gross description
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● Poorly demarcated (often receive in multiple fragments), gritty, tan-white, centered in soft tissue

Micro description
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● Fibromyxoid matrix with paucicellular, bland tumor cells in parallel or fascicular patterns
● Cells have dispersed chromatin, minimal nucleoli, eosinophilic or amphophilic cytoplasm
● Thick walled venule-sized vessels with open lumina
● No staghorn vessels
● No storiform growth, no nuclear pleomorphism, no / rare mitotic activity, no inflammatory infiltrate

Micro images
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A: fusiform cells; B: myxoid degeneration

Positive stains
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● Actin, desmin, vimentin

Electron microscopy description
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● Myofibroblastic features of intrareticular collagen fibers, thin filament bundles, cytoplasmic dense bodies

Differential diagnosis
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● Inflammatory myofibroblastic tumor
● Sclerosing mediastinitis

End of Mediastinum > Other benign / low grade tumors > Fibromatosis

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