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Soft Tissue Tumors

Fibrohistiocytic tumors

Giant cell tumor of tendon sheath - localized type


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 4 December 2013, last major update August 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Circumscribed proliferation of synovial like mononuclear cells with osteoclast-like giant cells, foam cells, siderophages and inflammatory cells, usually in digits

Terminology
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● Also called fibrous histiocytoma of tendon sheath, tenosynovial giant cell tumor-localized type, localized nodular tenosynovitis
● Family of lesions arising from joint synovium, bursae and tendon sheath; giant cell tumor of bone, giant cell tumor of tendon sheath and pigmented villonodular synovitis may have common cell lineage (Ultrastruct Pathol 2006;30:151)
● See also Joints chapter (fibrous histiocytoma of tendon sheath)

Epidemiology
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● Common, usually ages 30-50 years, 2/3 female, primarily affects the fingers
● #2 most common tumor of hand after ganglion cyst
● Rare in children, in whom recurrence is uncommon (J Pediatr Orthop 2007;27:67)

Clinical features
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● Slow growing, painless mass, 25 cm

Case reports
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● 9 year old girl with finger nodule (Dermatology Online Journal 2011;17:9)
● 9 year old boy with giant cell tumour with cutaneous satellites near surgical scar following excision of primary lesion (Case Rep Dermatol 2011;3:118)
● 22 year old man with acutely painful finger (AJR Am J Roentgenol 2007;188:S13)
● 29 year old woman and 30 year old man with ankle masses (J Med Case Rep 2009 Dec 15;3:9331)
● 37 year old woman with tumor intertwined with ulnar nerve (Eplasty 2009;9:e8)

Treatment and prognosis
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● Excision, 4-30% recur (treat with reexcision)

Clinical images
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9 year old girl with nodule in the right index finger


Tumors at surgery

Gross description
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● Localized tumors up to 4 cm in small joints (larger elsewhere), circumscribed, lobulated, white-gray-brown (yellow-orange-red color due to xanthoma cells and hemosiderin)
● May have shallow grooves along deep surface due to underlying tendons

Gross images
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Well circumscribed masses

Micro description
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● Well circumscribed, lobulated, partially encapsulated
● Variable proportions of mononuclear cells (small, round to spindled, with pale cytoplasm and round or grooved nuclei), osteoclast-like giant cells with 3-50 nuclei, foam cells, siderophages, epithelioid cells with glassy cytoplasm and round vesicular nuclei
● Varying amounts of dense collagenous stroma
● Up to 20 mitotic figures/10 HPF
● Stroma is hyalinized
● Cartilaginous metaplasia rarely seen

Micro images
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Ankle lesion


9 year old boy with hand lesion

Virtual slides
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Giant cell tumor of tendon sheath

Cytology description
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● High cellularity, polymorphic population of mononuclear histiocyte-like cells, hemosiderin laden macrophages, foamy macrophages and a few multinucleated giant cells (Acta Cytol 2008;52:152, Diagn Cytopathol 2004;30:14)
● Stromal cells are dispersed, polygonal to spindle shaped, with intranuclear cytoplasmic inclusions and occasional nuclear grooves and convolutions (Diagn Cytopathol 2003;29:105)

Cytology images
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Scattered groups of mononuclear cells with occasional osteoclast-like giant cells

Positive stains
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Mononuclear cells - CD68, variable muscle specific actin and desmin
Osteoclast-like giant cells - CD68, CD45 (Mod Pathol 1995;8:155), TRAP

Electron microscopy description
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● Histiocyte-like cells, fibroblast-like cells, multinucleated giant cells, foam cells

Molecular/cytogenetics description
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● Near diploid or pseudodiploid, often t(1;2) (Fetal Pediatr Pathol 2004;23:171)

Additional references
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eMedicine, Stanford University

End of Soft Tissue Tumors > Fibrohistiocytic tumors > Giant cell tumor of tendon sheath - localized type


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