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Soft Tissue Tumors

Fibrohistiocytic tumors

Giant cell tumor of soft tissue


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 27 October 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Soft tissue counterpart of giant cell tumor of bone (Mod Pathol 1999;12:894)
● Also called soft tissue giant cell tumor of low malignant potential (Stanford University)
● Prominent osteoclast-like giant cells and mononuclear cells, but at most mild atypia

Clinical features
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● Rare, usually age 40+ years, rare in children
● Usually superficial soft tissue of extremities (70%); rarely breast, mediastinum, groin, surgical scars
● Presents as painless growing mass

Case reports
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● 40 year old woman with nodule on thigh (Dermatology Online J 2010;16:2)
● 53 year old man with a mediastinal mass (Rare Tumors 2009 Dec 28;1:e45)
● 54 year old man with tumor of finger (Dermatol Online J 2008;14:7)
● 60 year old woman with breast tumor and fatal outcome (Ann Diagn Pathol 2007;11:345)
● 73 year old man with metastatic tumor associated with post-transplant immunosuppression (Virchows Arch 2006;448:847)
● Two cases arising in surgical scars (Pathologe 2009;30:401)
● Tumor of 46 years duration (J Cutan Pathol 2009;36:20)

Treatment and prognosis
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● Complete excision with negative margins
● Recurs in 12% (associated with incomplete excision)
● Rare metastases and death

Gross description
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● Up to 10 cm, usually dermis or subcutis, 30% are below superficial fascia
● Well circumscribed, nodular, fleshy, red-brown-gray, gritty at periphery due to calcification

Clinical images
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54 year old man


Large, smooth, firm, erythematous nodule on thigh

Gross images
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Mediastinal mass

Micro description
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● Multinodular with fibrous tissue containing siderophages separating nodules
● Nodules contain round/oval mononuclear cells and multinucleated osteoclast-like giant cells with similar nuclei
● Mononuclear cells are epitheloid or spindled, with eosinophilic cytoplasm and central nuclei
● Stroma is vascularized; up to 30 mitotic figures/10 HPF
● Often metaplastic bone, blood lakes, vascular invasion
● Occasionally fascicular pattern with focal storiform arrangement of spindle cells
● No/rare atypia, pleomorphism or necrosis

Micro images
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Resembles giant cell tumor of bone, with osteoclast-like giant cells but at most mild pleomorphism


Chondroid pattern   Osteoid pattern


Mediastinal mass


Thigh tumor


Osteoclast–like giant cells are CD68+ (lesion of pinna)


Bone tumor (for comparison): Numerous osteoclast-like giant cells and surrounding mononuclear cells


Bone tumor (for comparison): Ki67 staining in mononuclear cells

Cytology description
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● Numerous osteoclast-like giant cells and mononuclear cells with bland and vesicular nuclei
● Occasional/rare fragment of branching vasculature, mitotic figures (Acta Cytol 2003;47:1103)

Positive stains
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● Vimentin, CD68 (strong in multinucleated giant cells), smooth muscle actin
● Also alkaline phosphatase, osteoprotegerin, RANKL, TRAIL and TRAP (Hum Pathol 2005;36:945)

Molecular/cytogenetics description
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● Usually no cytogenetic abnormalities
● Case report of telomeric association involving multiple chromosomes (Pediatr Dev Pathol 2005;8:718)

Differential diagnosis
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Giant cell tumor of tendon sheath-localized: near tendons, hyalinized stroma, foam cells and hemosiderin laded macrophages are common, metaplastic bone is uncommon
Plexiform fibrohistiocytic tumor: childhood tumor with plexiform growth pattern, complex tentacle like extensions
Malignant fibrous histiocytoma-giant cell type: infiltrative, moderate to severe atypia of non-giant cells, necrosis, atypical mitotic figures

End of Soft Tissue Tumors > Fibrohistiocytic tumors > Giant cell tumor of soft tissue


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