Tenosynovial giant cell tumor: case report and review

Tenosynovial giant cell tumors are a group of generally benign intra-articular and soft tissue tumors with common histologic features. They can be roughly divided into localized and diffuse types. Localized types include giant cell tumors of tendon sheath and localized pigmented villonodular synovitis, whereas diffuse types encompass conventional pigmented villonodular synovitis and diffuse-type giant cell tumor. Localized tumors are generally indolent, whereas diffuse tumors are locally aggressive. Recent developments indicate that tenosynovial giant cell tumors are clonal neoplastic tumors driven by overexpression of CSF1. Herein, I report a case of intra-articular, localized tenosynovial giant cell tumor (or localized pigmented villonodular synovitis) and review the classification, histopathology, and recent developments regarding its pathogenesis.     

Giant cell tumor of bone express p63.

p63 contributes to skeletal development and tumor formation; however, little is known regarding its activity in the context of bone and soft tissue neoplasms. The purpose of this study was to investigate p63 expression in giant cell tumor of bone and to determine whether it can be used to discriminate between other giant cell-rich tumors. Seventeen cases of giant cell tumor of bone were examined to determine the cell type expressing p63 and identify the isoforms present. Total RNA or cell protein was extracted from mononuclear- or giant cell-enriched fractions or intact giant cell tumor of bone and examined by RT-PCR or western blot, respectively. Immunohistochemistry was used to evaluate p63 expression in paraffin embedded sections of giant cell tumor of bone and in tumors containing multinucleated giant cells, including: giant cell tumor of tendon sheath, pigmented villonodular synovitis, aneurysmal bone cyst, chondroblastoma, and central giant cell granuloma. The mononuclear cell component in all cases of giant cell tumor of bone was found to express all forms of TAp63 (alpha, beta, and gamma), whereas only low levels of the TAp63 alpha and beta isoforms were detected in multinucleated cells; DeltaNp63 was not detected in these tumors. Western blot analysis identified p63 protein as being predominately localized to mononuclear cells compared to giant cells. This was confirmed by immunohistochemical staining of paraffin-embedded tumor sections, with expression identified in all cases of giant cell tumor of bone. Only a proportion of cases of aneurysmal bone cyst and chondroblastoma showed p63 immunoreactivity whereas it was not detected in central giant cell granuloma, giant cell tumor of tendon sheath, or pigmented villonodular synovitis. The differential expression of p63 in giant cell tumor of bone and central giant cell granuloma suggest that these two tumors may have a different pathogenesis. Moreover, p63 may be a useful biomarker to differentiate giant cell tumor of bone from central giant cell granuloma and other giant cell-rich tumors, such as giant cell tumor of tendon sheath and pigmented villonodular synovitis.     

Malignant giant cell tumor of synovium and locally destructive pigmented villonodular synovitis: ultrastructural and immunohistochemical study and review of the literature

The first reported case of an intraarticular malignant giant cell tumor of synovium studied with electron microscopic and immunohistochemical examination is presented, together with a case of diffuse intraarticular pigmented villonodular synovitis with extensive bone destruction. The malignant case was dominated by uniform cells positive for histiocytic markers, the fine structure showing a gradual change from cells dominated by organelles serving a secretory function to cells with phagocytic activity. The reported cases of giant cell tumor of the tendon sheath indicate that the pertinent histologic changes regarding malignancy are an increase in cell polymorphism and in the number of mitoses, and a decrease in the number of multinucleated giant cells.     

Giant cell tumor of tendon sheath and pigmented villonodular synovitis: an ultrastructural study

The ultrastructures of five examples of giant cell tumors of the tendon sheath and one example of pigmented villonodular synovitis were studied. The giant cell tumors were composed mostly of A and B types of synovial cells. Also present were fibroblastic cells with intracytoplasmic filaments with densities (myofibroblasts) as well as "monocytic" and "lymphocytic" cells. The giant cells seemingly were derived from the fusion of synovial cells of A type, whereas foam cells were derived from both A and B cells. Both the giant cell tumors and the pigmented synovitis are considered to be reactive and borderline proliferative lesions of the synovial cells.     

Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathologic and flow cytometric DNA analysis.

The DNA content and proliferative indexes of seven cases of tenosynovial giant cell tumor of tendon sheath, diffuse type (TGCT-D); 11 cases of tenosynovial giant cell tumor of tendon sheath, localized type (TGCT-L); and seven cases of pigmented villonodular synovitis (PVNS) were analyzed by flow cytometry in an attempt to assess objectively their biologic differences. Three cases of TGCT-D manifested an aneuploid DNA content and four had a diploid DNA pattern. All cases of TGCT-L and PVNS showed a diploid DNA content. The proliferative indexes for TGCT-D were significantly higher than those found in the other two groups. There was no histopathologic feature that correlated with the aneuploid DNA pattern found in two of the three cases of TGCT-D. Only one of the three aneuploid DNA content TGCT-D cases displayed marked cellular pleomorphism with dense fibrous stroma; in that case there was recurrence 4 years after initial excision. Our data further support that TGCT-D, TGCT-L, and PVNS are histopathologically similar but clinically distinct lesions. The high proliferative indexes of TGCT-D may reflect a rapid, uncontrolled growth that may explain its aggressive biologic behavior. The presence of an aneuploid DNA pattern in some cases of TGCT-D in this study, coupled with the reported chromosomal abnormalities and occurrence of malignant transformation in these lesions, clearly supports their neoplastic nature.     

Malignant giant cell tumor of tendon sheath. Report of a case

In a patient with pigmented villonodular synovitis of the right knee joint, there occurred a malignant giant cell tumor of tendon sheath. There was clinical evidence of metastasis after the second local recurrence and the recurrent tumors were studied enzyme cytochemically and electron microscopically. Ultrastructurally, the malignant tumor consisted of three principal cell types; histiocyte-like cells, fibroblast-like cells, and intermediate cells, with unique attendance of myofibroblasts. This may be the first report of the presence of myofibroblasts in malignant giant cell tumor of tendon sheath. Enzyme cytochemistry revealed various functional properties of histiocytes.     

MALIGNANT GIANT CELL TUMOR OF TENDON SHEATH Report of a Case

In a patient with pigmented villonodular synovitis of the right knee joint, there occurred a malignant giant cell tumor of tendon sheath. There was clinical evidence of metastasis after the second local recurrence and the recurrent tumors were studied enzyme cytochemically and electron microscopically. Ultrastructurally, the malignant tumor consisted of three principal cell types; histiocyte-like cells, fibroblast-like cells, and intermediate cells, with unique attendance of myofibroblasts. This may be the first report of the presence of myofibroblasts in malignant giant cell tumor of tendon sheath. Enzyme cytochemistry revealed various functional properties of histiocytes. ACTA PATHOL. JPN. 35 : 699–709, 1985.     

Pathology of the synovium

Synovium is specialized mesenchymal tissue that is essential for the appropriate function of the locomotor apparatus. It is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. In this article, the normal microscopic anatomy of synovium is briefly reviewed. Synovial proliferative disorders, including pigmented villonodular synovitis, giant cell tumor of tendon sheath, hemosiderotic synovitis, and fatty infiltration of the synovial membrane are discussed. Additionally, the subjects of intrasynovial cartilaginous lesions (primary and secondary synovial chondromatosis) and crystal deposition diseases are reviewed. Finally, the response of synovial tissues to implanted foreign materials that are used in large and small joint arthroplasty are described.     

Tenosynovial giant cell tumor arising from the posterior cruciate ligament: a case report and literature review

The localized form of tenosynovial giant cell tumor or pigmented villonodular synovitis is rarely intraarticular in the knee. We reported a 40-year-old woman with a tenosynovial giant cell tumor arising from posterior cruciate ligament (PCL). She suffered sudden knee pain and locking without any reason for two days. A mass with a size of 1.7×0.8×0.7 cm in the fossa intercondyloidea was detected on the MRI. After one time hyperextension physical examination the patients felt sudden pain relief. During the arthroscopy examination, a loose soft tissue mass was found under the lateral meniscus. Only the synovium tissue lesion on the proximal PCL was detected. The mass had a conceivable thin pedicel and the shape matched well with the tumor bed on the PCL. The histopathology of the mass demonstrated a tenosynovial giant cell tumor. At six weeks follow-up, no clinical evidence of recurrence was noted. A Literature Review of tenosynovial giant cell tumor or pigmented villonodular synovitis arising from the PCL is present.     

Pigmented villonodular synovitis (giant cell tumor of the synovium) occurring in the vertebral column. Report of a case.

A case of pigmented villonodular synovitis (giant cell tumor of the synovium) involving the vertebral column is presented. The tumor grew outside the dura and extended to the paravertebral connective tissue, causing sensory and motor disturbance indicative of spinal cord compression. This anatomic location is very rare for lesions of this type, and to our knowledge, this case is only the fifth reported in the English-language literature.     

Pigmented Villonodular Synovitis (Giant Cell Tumor of the Synovium) Occurring in the Vertebral Column

A case of pigmented villonodular synovitis (giant cell tumor of the synovium) involving the vertebral column is presented. The tumor grew outside the dura and extended to the paravertebral connective tissue, causing sensory and motor disturbance indicative of spinal cord compression. This anatomic location is very rare for lesions of this type, and to our knowledge, this case is only the fifth reported in the English language literature.     

Tenosynovialer Riesenzelltumor

Morphological, ultrastructural, and immunohistochemical findings of 12 diffuse type-tenosynovial giant cell tumors/pigmented villonodular synovitis are presented compared to 30 localized tenosynovial giant cell tumors (giant cell tumor of tendon sheath). Diffuse-type-tenosynovial giant cell tumor is characterized by a striking vascularisation pattern composed of densely arranged thin-walled, partly slit-like and partly hyalinized small blood vessels within the papillary synovial fronds. These vessels may show abnormal structures with incompletely arranged endothelial cells/pericytes. The fibrohistiocytic tumor cells probably cause considerable compression/distortion or destruction of the small vessels which might be responsible for an increased blood deposition and massive hemosiderosis. Accompanying multinucleated osteoclast-like giant cells seemingly are recruited from circulating blood monocytes. Microhemorrhagic foci with multinucleated giant cells could be detected in 83% of diffuse-type and 67% of localized-type tumors. Apart from the described vessels, typical morphological findings in diffuse-type tenosynovial giant cell tumors included "giant" hemosiderotic granules, (at least 2-3 times the diameter of an erythrocyte) "giant" siderophages, pseudoalveolar clefts and irregularly anastomosing synovial fronds. Neither mitotic rate nor the amount of giant cells/amount of nuclei of giant cells revealed statistically significant differences between localized-type and diffuse-type of tenosynovial giant cell tumor. Immunohistochemically, the diffuse-type exhibited focal expression of CD31 (in 75% of tumors) and calretinin (in 63%) besides CD68-staining.     

Similarities between giant cell tumor of bone, giant cell tumor of tendon sheath, and pigmented villonodular synovitis concerning ultrastructural cytochemical features of multinucleated giant cells and mononuclear stromal cells

The authors investigated ultrastructural cytochemical features of multinucleated and mononuclear stromal cells in giant cell tumor of bone (GCTB), giant cell tumor of tendon sheath (GCTTS), and pigmented villonodular synovitis (PVNS). Specimens of each tumor, respectively numbering 4, 4, and 3, were stained for tartrate-resistant acid phosphatase (TRAP) reactions and examined with an electron microscope. In GCTB and GCTTS, multinucleated cells, including some relatively small giant cells, showed TRAP activity and cytoplasmic features characteristic of osteoclasts, and also sometimes abundant rough endoplasmic reticulum and siderosomes. A few giant cells with macrophage-like features and slight TRAP activity were demonstrated in GCCTS and PVNS. In each tumor type, mononuclear cells showing TRAP activity shared cytoplasmic features with osteoclast-like multinucleated giant cells, while some others had macrophage-like features, and still others were poorly differentiated; a few mononuclear cells showed cell-to-cell contact. Ultrastructural similarities of TRAP-positive mononuclear cells in the three tumor types, and those between TRAP-positive multinucleated cells in GCTB and GCTTS, suggest a common cell lineage capable of multinucleated giant cell formation in the 3 tumors, despite differing histogenesis.     

Diffuse-type giant cell tumor/pigmented villonodular synovitis arising in the sacrum: malignant form

Diffuse-type giant cell tumor (GCT)/pigmented villonodular synovitis (PVNS) in the axial skeleton or spine is rare. Herein is reported a case of diffuse-type GCT/PVNS involving the sacrum and the fifth lumbar vertebra, in which the patient developed regional lymph node swelling after recurrence. The recurrent tumor was found to have atypical histological features such as spindle cell morphology, cytological atypia and high mitotic rate, which are compatible with the diagnostic criteria of secondary malignant diffuse-type GCT/PVNS. Although the nodal lesions were not sampled histologically, the clinical and histological features indicate that the current case is an example of malignant diffuse-type GCT/PVNS. This case is considered to be the first case of malignant diffuse-type GCT/PVNS in the spine, because no such lesions have been previously reported in the axial skeleton or spine. Careful surveillance should be required for diffuse-type GCT/PVNS arising at unusual site.     

Diffuse pigmented villonodular synovitis of the temporomandibular joint diagnosed by fine-needle aspiration cytology

Diffuse pigmented villonodular synovitis is a rare tumor in the temporomandibular joint region. This article deals with a 32-yr-old male who suffered from pain and swelling in the right temporomandibular joint region associated with restricted mouth opening. Computed tomography showed a tumor lateral to the temporomandibular joint. Arthrography revealed a displaced temporomandibular joint disk. Fine-needle aspiration cytology showed characteristic cellular changes, including rounded or oval cells with abundant cytoplasm and intracytoplasmatic hemosiderin deposits and numerous multinucleated giant cells without nuclear atypia. A benign mesenchymal lesion suggestive for pigmented villonodular synovitis was diagnosed and later verified at histologic examination. Fine-needle aspiration cytology seems to be useful for this diagnosis.     

Pigmented villonodular synovitis

Pigmented villonodular synovitis (PVNS) is a destructive proliferative tumoroid disease of the synovial sheath of the joints, bursae, and tendon. The microscopic pattern of PVNS is characterized by mosaicism: compact cell zones alternate with loose few-celled fields. The synovial sheath is hyperplastic; its stroma exhibits hemosiderin and siderophages, as well as histiocytes, lymphocytes, multinucleated giant osteoclasts, foamy and synoviocyte-like cells. Proliferation with destructive growth was noted in PVNS. A distinction is made between focal and diffuse, intraarticular and extraarticular forms of the disease. Early diagnosis of the disease favors effective treatment and prevention of soft tissue and bone destruction and reduces the risk of recurrences.     

Pigmented villonodular synovitis with chondroid metaplasia, resembling chondroblastoma of the bone: a report of three cases.

We herein describe three cases of pigmented villonodular synovitis with chondroid metaplasia. Two cases involved the temporomandibular joint, whereas the remaining one case occurred in the hip joint. Histologically, the tumors showed a villous pattern and were mainly composed of histiocyte-like cells and scattered osteoclast-like multinucleated giant cells, accompanied by chondroid areas with occasional lace-like calcification. These features resembled those of chondroblastoma of the bone, with the exception of the villous pattern. The histiocyte-like cells showed positive immunoreactivity for CD68, whereas they were negative for S-100 protein. Some of the previously reported cases of chondroblastoma in the temporal bone may have actually been cases of pigmented villonodular synovitis with chondroid metaplasia. When histologically chondroblastoma-like lesions involve the temporal bone or temporomandibular joint, the possibility of pigmented villonodular synovitis with chondroid metaplasia should also be considered, in addition to chondroblastoma of the bone. The correlation between this lesion and synovial chondromatosis remains uncertain.     

Pigmented villonodular synovitis secondary to laceration of the perforating branch of the peroneal artery

A case of peroneal artery injury subsequently developed into a lesion resembling an extra-articular tenosynovial giant cell tumor, which is a type of pigmented villonodular synovitis (PVNS). This case supports the hypothesis that accident trauma, such as a vascular injury, can be the etiology of PVNS.