diffuse giant cell tumor of tendon sheath
Wednesday 18 February 2004
pigmented villonodular synovitis; pigmented villonodular tenosynovitis; tenosynovial giant cell tumor, diffuse type; diffuse type of giant-cell tumor of the tendon sheath (GCTTS); tenosynovial giant cell tumor-diffuse type; formerly called pigmented villonodular synovitis (PVNS) when occurs near a joint; giant cell tumor of tendon sheath, diffuse type
JRC:6551 : Pigmented villonodular synovitis.
Definition: Diffuse giant cell tumor of tendon sheath (pigmented villonodular synovitis) is an extra-articular, destructive villonodular hyperplasia with synovial mononuclear cells mixed with multinucleated giant cells, foam cells, siderophages and inflammatory cells.
In these less common diffuse form of giant cell tumor of tendon sheath, the knee and ankle joints are commonly affected with a fond papillary synovitis and a histology similar to the nodular type.
The diffuse form of giant cell tumor of tendon sheath is not encapsulated but, instead, grows around the joint cavity with extension to soft tissue. These lesions are much more locally aggressive, eroding joints and recurring in nearly half of the cases.
The diffuse form of giant cell tumor of tendon is considered the soft tissue counterpart of "pigmented villonodular synovitis". It may represent extension of articular tumor since often occurs near a joint
Rare; less frequent than localized type
Usually < age 40 years
Develops in synovial lining of joints, tendon sheaths and bursae
Usually knee (80%); also ankle, hip, shoulder or elbow joint
Almost always monoarticular
Occasionally invades underlying bone; may cause bone cyst formation, loss of bone and cartilage
Brown-yellow spongy tissue, firm and nodular, often 5 cm or larger
arise in the vicinity of large joints of the lower extremity
proliferation of polygonal mononuclear cells
osteoclastlike giant cells
infiltrative pattern into the neighboring soft tissues
Its anatomic distribution parallels that of PVNS, with lesions most commonly found around the knee, followed by the ankle and foot; however, the diffuse form occasionally affects the hand.
Typically, these lesions, like those of PVNS, occur in young patients; 50% of cases are diagnosed in patients younger than 40 years.
The diffuse form is often locally aggressive, and multiple recurrences after excision are common.
Because of the similarities in age, tumor locations, clinical presentations, and symptoms for patients with PVNS and patients with the diffuse form of giant cell tumors of the tendon sheath, the diffuse form probably represents an extra-articular extension of a primary intra-articular PVNS process.
Findings from flow cytometric DNA analysis suggest that PVNS and giant cell tumors of the tendon sheath are histopathologically similar but clinically distinct lesions.
When the origin of these poorly confined soft-tissue masses is uncertain, Enzinger and Weiss classify these tumors as the diffuse type of giant cell tumors of the tendon sheath, whether or not they involve the adjacent joint.
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
Round, spindled and multinucleated cells without atypia
Round cells have minimal cytoplasm and eccentric nuclei
occasional cytoplasmic hemosiderin
no mitotic figures
strong expression of vimentin, neuron-specific enolase, A1-antitrypsin, and CD68 in both mono- and multinucleated tumor cells.
mononuclear cells : diverse morphology, features of histiocytelike and fibroblastlike cells, with the former being more numerous.
scarce neurosecretorylike granules, made up of electrondense membrane-bound material, in the cytoplasm of the mononuclear cells.
der(8) t(8;12) (#12028654#)
only rarely has malignant behavior
Treatment and prognosis
Excision, may recur if inadequate excision;
radiation therapy for recurrences
giant cell tumor of the tendon sheath (GCTTS)
malignant forms of diffuse type of GCTTS (HP.)
Ferrer J, Namiq A, Carda C, López-Ginés C, Tawfik O, Llombart-Bosch A. Diffuse type of giant-cell tumor of tendon sheath: an ultrastructural study of two cases with cytogenetic support. Ultrastruct Pathol. 2002 Jan-Feb;26(1):15-21. PMID: #12028654#
Diffuse Type of Giant-Cell Tumor of Tendon Sheath: An Ultrastructural Study of Two Cases With Cytogenetic Support. Ferrer J; Namiq A; Carda C; López-Ginés C; Tawfik O; Llombart-Bosch A. Ultrastructural Pathology, Volume 26, Number 1, 1 January 2002 , pp. 15-21(7)