Primary tumours of the synovium

Bhadra, Arup K.; Pollock, Rob; Tirabosco, Roberto; Skinner, John; Cannon, Steve; Briggs, Tim; Flanagan, Adrienne M.

doi:10.1302/0301-620x.89b11.18963

Cited by 28 publications

(7 citation statements)

References 25 publications

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“…[ 4 7 ] Criteria for malignant TGCT were also developed at the Armed Forces Institute of Pathology (AFIP) to include five out of eight characteristics including diffuse pleomorphism, prominent nucleoli, high cytoplasmic to nuclear ratios, mitotic ratio greater than 10 per 10 HPFs, necrosis, discohesion of tumor cells, paucity of giant cells, and a diffuse growth pattern. [ 8 ] Our case demonstrated features described by both Bertoni et al, and the AFIP to be classified as malignant [ Figure 5b ] and was confirmed by histologic evaluation to be metastatic to inguinal lymph nodes. The initial biopsy of the lesion was atypical including a mitotic rate greater than 10 per 10 HPFs as well as necrosis; however, it did not fulfill enough of the criteria to be classified as malignant TGCT.…”

Section: Discussionsupporting

confidence: 79%

Malignant Tenosynovial Giant Cell Tumor of the Leg: A Radiologic-Pathologic Correlation and Review of the Literature

Richman

Bresler

Rosenthal

et al. 2015

Journal of Clinical Imaging Science

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Malignant tenosynovial giant cell tumor (TGCT) is a rare clinical entity that can arise as a recurrent lesion or can co-exist with a benign TGCT lesion. Malignant TGCT most commonly arises in the lower extremity and tends to be clinically aggressive, with most patients developing recurrent lesions or dying. Much of the literature describes the histopathologic features and classifies this broad group of tumors, with little description of the imaging characteristics of this disease. We present the multimodality appearance of a case of malignant diffuse-type TGCT that recurred 2 months after resection with subsequent rapid clinical progression.

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Section: Discussionsupporting

confidence: 79%

Malignant Tenosynovial Giant Cell Tumor of the Leg: A Radiologic-Pathologic Correlation and Review of the Literature

Richman

Bresler

Rosenthal

et al. 2015

Journal of Clinical Imaging Science

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“…In some cases a gradient of differentiation (zoning phenomenon) between the periphery and the central part of the nodules was observed [ 5 ]. To our knowledge, 32 cases of malignant D-TCGT are reported in literature: 17 cases were primary malignant D-TGCT and 15 arose from a prior histologically benign lesion [ 1 – 6 , 9 ]. Seven out of these 32 cases metastasized to regional lymph node: four patients died of disease with a rapid progression (mean 22 months, range 12–41 months), two were alive with disease 17, and 36 months after the first surgical treatment, respectively and only one patient was well without disease 10 months after the first surgical treatment.…”

Section: Discussionmentioning

confidence: 99%

Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis

et al. 2015

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Tenosynovial giant cell tumour, diffuse type, also known under a variety of other terms including diffuse pigmented villonodular synovitis, tends to be locally aggressive and not infrequently can show multiple recurrences. The differential diagnosis with the extremely rare and somewhat controversial malignant variant of tenosynovial giant cell tumour, diffuse type, is challenging due to overlapping radiologic features of these two entities. Malignant tenosynovial giant cell tumour is defined by the presence of overtly malignant sarcomatous areas. We describe a very unusual case of a 63-year-old man affected by tenosynovial giant cell tumour, diffuse type of the knee that, despite absence of morphologic evidence of sarcomatous transformation, developed inguinal lymph node metastases following multiple surgical procedures.

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“…Bertoni et al [13] defined these lesions in somewhat less strict manner, including tumors showing morphological features similar to malignant tenosynovial giant cell tumor even without simultaneous or prior tenosynovial giant cell tumor. Malignant tenosynovial giant cell tumors are extremely rare and relatively poorly understood [4,5,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28].…”

Section: Introductionmentioning

confidence: 99%

Malignant Tenosynovial Giant Cell Tumor: The True “Synovial Sarcoma?” A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes

et al. 2019

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We present our experience with ten well-characterized malignant tenosynovial giant cell tumors, including detailed immunohistochemical analysis of all cases and molecular cytogenetic study for CSF1 rearrangement in a subset. Cases occurred in 7 M and 3 F (mean age: 52 years; range: 26-72 years), and involved the ankle/foot (n = 1), finger/toe (n = 3), wrist (n = 1), pelvic region (n = 3), leg (n = 1), and thigh (n = 1). There were eight primary and two secondary malignant tenosynovial giant cell tumors. Histologically, all cases showed definite areas of typical tenosynovial giant cell tumor. The malignant areas varied in appearance. In some cases, isolated malignant-appearing large mononuclear cells with high nuclear grade and mitotic activity were identified within otherwise-typical tenosynovial giant cell tumor, as well as forming larger masses of similar-appearing malignant cells. Occasionally, these nodules of malignant large mononuclear cells showed transition to pleomorphic spindle cell sarcoma, with varying degrees of collagenization and myxoid change. One malignant tenosynovial giant cell tumor was composed of sheets of monotonous large mononuclear cells with high nuclear grade, growing in a hyalinized, osteoid-like matrix, with areas of heterologous osteocartilaginous differentiation. Mitotic activity ranged from 2 to 34 mitoses per 10 HPF (mean 18/10 HPF). Geographic necrosis was observed in four cases. The malignant-appearing large mononuclear cells were consistently positive for clusterin and negative for CD163, CD68, and CD11c. Desmin was positive in a small minority of these cells. Areas in malignant tenosynovial giant cell tumor resembling pleomorphic spindle cell sarcoma or osteo/chondrosarcoma showed loss of clusterin expression. RANKL immunohistochemistry was positive in the large mononuclear cells in eight cases. Two cases showed an unbalanced rearrangement of the CSF1 locus. Follow-up (nine patients; range 0.5-66 months; mean 20 months) showed three patients dead of disease, with three other living patients having lung and lymph node metastases; three patients were disease-free. We conclude that malignant tenosynovial giant cell tumors are highly aggressive sarcomas with significant potential for locally destructive growth, distant metastases, and death from disease. The morphologic and immunohistochemical features of these tumors and the presence of CSF1 rearrangements support origin of malignant tenosynovial giant cell tumor from synoviocytes.

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Primary tumours of the synovium

Abstract: Four patients who developed malignant synovial tumours are described; one with chondromatosis developed a synovial chondrosarcoma and three with pigmented villonodular synovitis developed malignant change. The relevant literature is discussed.

Cited by 28 publications

References 25 publications

Malignant Tenosynovial Giant Cell Tumor of the Leg: A Radiologic-Pathologic Correlation and Review of the Literature

Malignant Tenosynovial Giant Cell Tumor of the Leg: A Radiologic-Pathologic Correlation and Review of the Literature

Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis

Malignant Tenosynovial Giant Cell Tumor: The True “Synovial Sarcoma?” A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes

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