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Soft Tissue Tumors
Giant cell tumor of tendon sheath - diffuse 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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● Extra-articular, destructive villonodular hyperplasia with synovial mononuclear cells mixed with multinucleated giant cells, foam cells, siderophages and inflammatory cells
● Considered the soft tissue counterpart of pigmented villonodular synovitis - may represent extension of articular tumor since often occurs near a joint
Terminology
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● Also called tenosynovial giant cell tumor-diffuse type
● Called pigmented villonodular synovitis (PVNS) when occurs near a joint
● Note: terminology of giant cell tumors is inconsistent in literature
Epidemiology
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● Rare; less frequent than localized type
● Usually < age 40 years
Clinical
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● Develops in synovial lining of joints, tendon sheaths and bursae
● Usually knee (80%); also ankle, hip, shoulder or elbow joint
● Almost always monoarticular
● May actually be hyperplastic, not neoplastic (Hum Path 2003;34:65)
● Occasionally invades underlying bone; may cause bone cyst formation, loss of bone and cartilage
● Locally aggressive; often recurs, but only rarely has malignant behavior (see below)
Case reports
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● 26 year old woman with tumor of subcutaneous thigh (Skeletal Radiol 2007;36:327)
● Arising in bursa of knee (Knee 2007;14:402)
Treatment and prognosis
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● Excision, may recur if inadequate excision; radiation therapy for recurrences
● See below for cases with malignant behavior
Gross description
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● Brown-yellow spongy tissue, firm and nodular, often 5 cm or larger
Gross images
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Knee synovectomy shows Multinodular red-brown White gray cartilage
diffuse involvement with tumor (Univ Alabama) (arrow) and brown villous
villous areas and a few nodules; tissue
flat shiny surface is normal synovium
Micro description
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● Diffuse expansive sheets of cells with infiltrative borders and variable cellularity
● Also hyperplastic synovium with papillary projections composed of foamy histiocytes and hemosiderin containing macrophages
●;Large clefts, pseudoglandular or alveolar spaces lined by synovial cells, osteoclast-like multinucleated (10-70 nuclei) giant cells, epithelioid cells
● Abundant collagen may be present, but lymphocytes and plasma cells are sparse
● Also giant hemosiderotic granules (2-3x diameter of RBC), giant siderophages (Pathologe 2005;26:96)
● May have 5+ mitotic figures/10 HPF, rarely chondroid metaplasia (Mod Path 2007;20:545)
● Malignant if nodular and solid invasive growth plus large cells with large nuclei, prominent nucleoli, necrotic areas and atypical mitotic figures (see below)
Micro images
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Villi have cellular cores, Villi lined by synovial cells and Various images
variable width and shape histiocytes; giant cells and
lymphocytes are also present
Papillary tumor Multinucleated giant cells, CD68+, S100+
synovial cells, fibroblasts cells in chondroid
and inflammatory cells areas
Multiple synovial lined clefts and hemosiderin deposits
in the subsynovial layer
Other images: shoulder tumor #1; #2; #3; #4; #5; histiocytes and giant cells in dense connective tissue stroma #1; #2; iron deposits (Prussian blue stain); foam cells
Cytology description
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● Round, spindled and multinucleated cells without atypia
● Round cells have minimal cytoplasm and eccentric nuclei, occasional cytoplasmic hemosiderin, no mitotic figures
Cytology images
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Diff-Quick and H&E
Positive stains
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● CD68 (stromal and giant cells, Hum Path 2003;34:65),
●
Also CD31 (75%), calretinin (63%, Pathologe 2005;26:96), desmin (35-40%)
Negative stains
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● S100 (but chondroid areas are S100+), CD45/LCA, EMA, keratin, HMB45, CD34, smooth muscle actin
Electron microscopy
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● Features of histiocytes and fibroblasts (Ultrastruct Pathol 2002;26:15)
Molecular / cytogenetics
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● Neoplastic and reactive features
● Neoplastic due to CSF1 overexpression (AJSP 2007;31:970) or 1p13 (CSF1) rearrangements, often with COL6A3 at 2q35 (Proc Natl Acad Sci USA 2006;103:690)
● Also reactive features (Hum Path 2003;34:65)
Differential diagnosis
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● Hemosiderotic synovitis - associated with hemophilia and intraarticular bleeding, no mononuclear or giant cell nodular proliferation, hemosiderin primarily in synovial lining cells
Additional references
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● eMedicine #1, #2, AAFP
Malignant giant cell tumor of tendon sheath-diffuse type
Definition
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● Rare tumor with coexisting or prior benign giant cell tumor plus sarcomatous areas
● Not a WHO diagnosis
Clinical
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● Mean 61 years, no gender preference
● Usually extra-articular, near large joints or extremities
● Often metastasizes or recurs, but variable outcome (AJSP 2008;32:587, Zhonghua Wai Ke Za Zhi 2008;46:1645)
Case reports
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● 72 year old woman with hip tumor (Jpn J Clin Oncol 1993;23:317)
Gross description
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● Mean 9 cm
Micro description
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● Benign areas with gradual or abrupt change to frank sarcoma containing pleomorphic, spindle or enlarged oval cells resembling MFH, fibrosarcoma, myxosarcoma or giant cell tumor with large nuclei and prominent nucleoli (AJSP 1997;21:153)
● Also necrosis and atypical mitotic figures
Micro images
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Benign and malignant areas
Molecular cytogenetics
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● Malignant cases may have trisomy 5 and 7 (Archives 2000;124:1636)
End of Soft Tissue Tumors > Giant cell tumor of soft tissue
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