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Soft Tissue Tumors
Giant cell tumor of tendon sheath - localized type
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Reviewer: David Lucas, M.D., University of Michigan Health Systems (January 2009)
Revised: 26 June 2009, last major update June 2009
Definition
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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
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)
Case reports
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● 22 year old man with acutely painful finger (AJR Am J Roentgenol 2007;188:S13)
● 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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Tumor entangled in Tumor extension into Nodular tumor of middle
ulnar nerve carpal tunnel finger
Gross description
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● Localized tumors are 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 mass Well circumscribed brown mass
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
● Up to 20 mitotic figures/10 HPF
● Stroma is hyalinized
Micro images
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Various images Nodular lesion of finger Small histiocytes and multi-
nucleated giant cells in dense
fibrous stroma
Virtual slide (Univ Iowa)
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)
Positive stains
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● Mononuclear cells - CD68, variable muscle specific actin and desmin
● Osteoclast-like giant cells - CD68, CD45 (Mod Path 1995;8:155), TRAP
Electron microscopy
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● Histiocyte-like cells, fibroblast-like cells, multinucleated giant cells, foam cells
Molecular / cytogenetics
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● Near diploid or pseudodiploid, often t(1;2) (Fetal Pediatr Pathol 2004;23:171)
Additional references
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End of Soft Tissue Tumors > Giant cell tumor of tendon sheath - localized type
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