Tenosynovial giant cell tumor: case report and molecular investigation.

Tenosynovial giant cell tumor is a benign neoplasm arising from the synovium of joints, including the temporomandibular joint (TMJ). Despite its benign nature, these tumors may exhibit aggressive behavior. A 57-year-old woman with a swollen, hardened area in the left TMJ was referred to the university´s clinic. The diagnosis of tenosynovial giant cell tumor was made based on the presence of hyperplastic synovial lining containing mononuclear and giant cells, hemorrhagic areas, hemosiderin deposits, and calcification foci in the biopsy. A low condylectomy was performed, and histopathologic analysis of the surgical piece upheld the diagnosis. Due to histopathologic resemblance with other giant cell-rich lesions (giant cell granuloma of the jaws, brown tumor of hyperparathyroidism, and non-ossifying fibroma) for which signature mutations are known, mutational analysis of KRAS, FGFR1, and TRPV4 genes was conducted. The results revealed wild-type sequences for all the mutations tested, thereby supporting the diagnosis of tenosynovial giant cell tumor.

Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report.

Tenosynovial giant cell tumor of diffuse type (TGCT-d) or pigmented villonodular synovitis (PVNS) is a locally aggressive lesion that mostly affects the joints of long bones. Chondroid tenosynovial giant cell tumor (CTGCT) or PVNS with chondroid metaplasia is a rare distinct subset of synovial tumors that has a predilection for the TMJ. We report a rare case of CTGCT in the TMJ, initially misdiagnosed as temporomandibular disorder (TMD). A 51-year-old woman was referred to the surgeon with the chief complaint of TMJ pain for 5 years and a past history of an unsuccessful TMD treatment. Extraoral examination revealed discrete preauricular swelling and restricted mandibular range of motion. Panoramic radiograph and computerized tomography showed destruction of the mandibular fossa and condyle. Histologically, the tumor was composed by large mononuclear cells with prominent eosinophilic cytoplasm and grooved nuclei, small histiocytoid cells, osteoclast-like multinucleated cells, brown pigmentation and areas of chondroid metaplasia. Morphological and immunohistochemical characteristics lead to the final diagnosis of CTGCT. The rarity of CTGCT could be attributed to the lack of recognition of this lesion, with cases diagnosed as chondroblastomas, synovial chodromatosis and chondrosarcoma. The patient received immediate reconstruction and recurrence was found 22 months after initial intervention. TGCT-d and CTGCT of the TMJ can present similar symptoms to TMD, but clinicians must distinguish both lesions by complete examination, imaging and, when necessary, histopathologic evaluation.

Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report

Abstract Tenosynovial giant cell tumor of diffuse type (TGCT-d) or pigmented villonodular synovitis (PVNS) is a locally aggressive lesion that mostly affects the joints of long bones. Chondroid tenosynovial giant cell tumor (CTGCT) or PVNS with chondroid metaplasia is a rare distinct subset of synovial tumors that has a predilection for the TMJ. We report a rare case of CTGCT in the TMJ, initially misdiagnosed as temporomandibular disorder (TMD). A 51-year-old woman was referred to the surgeon with the chief complaint of TMJ pain for 5 years and a past history of an unsuccessful TMD treatment. Extraoral examination revealed discrete preauricular swelling and restricted mandibular range of motion. Panoramic radiograph and computerized tomography showed destruction of the mandibular fossa and condyle. Histologically, the tumor was composed by large mononuclear cells with prominent eosinophilic cytoplasm and grooved nuclei, small histiocytoid cells, osteoclast-like multinucleated cells, brown pigmentation and areas of chondroid metaplasia. Morphological and immunohistochemical characteristics lead to the final diagnosis of CTGCT. The rarity of CTGCT could be attributed to the lack of recognition of this lesion, with cases diagnosed as chondroblastomas, synovial chodromatosis and chondrosarcoma. The patient received immediate reconstruction and recurrence was found 22 months after initial intervention. TGCT-d and CTGCT of the TMJ can present similar symptoms to TMD, but clinicians must distinguish both lesions by complete examination, imaging and, when necessary, histopathologic evaluation.

Resumo Tumor de células gigantes tenossinovial do tipo difuso (TCGT-d) ou sinovite vilonodular pigmentada (SVP) é uma lesão localmente agressiva que afeta principalmente as articulações dos ossos longos. Tumor de células gigantes tenossinovial condroide (TCGTC) ou SVP com metaplasia condroide é um tipo distinto e raro de tumor sinovial que tem a predileção pela articulação temporomandibular (ATM). Nós relatamos um caso raro de TCGTC da ATM, inicialmente diagnosticado, equivocadamente, como disfunção temporomandibular (DTM). Uma mulher de 51 anos foi encaminhada ao cirurgião com a queixa principal de dor na ATM por 5 anos, e uma história de tratamento de DTM sem sucesso. O exame extrabucal revelou discreto aumento de volume preauricular e movimentação mandibular restrita. A radiografia panorâmica e a tomografia computadorizada evidenciaram destruição da fossa mandibular e côndilo. Histologicamente, o tumor era composto por células mononucleares grandes, com amplo citoplasma eosinofílico e núcleo sulcado, pequenas células histiocitoides, células multinucleadas semelhantes a osteoclastos, pigmentação acastanhada e áreas de metaplasia condroide. As características morfológicas e imuno-histoquímicas levaram ao diagnóstico final de TCGTC. A raridade desta lesão pode estar associada ao seu não reconhecimento, sendo casos diagnosticados como condroblastoma, condromatose sinovial ou condrossarcoma. A paciente recebeu reconstrução imediata e recorrência foi observada 22 meses após a intervenção inicial. TCGT-d e TCGTC da ATM podem apresentar sintomas similares à DTM, mas os clínicos devem diferenciar ambas as lesões por meio do exame clínico completo, exames de imagem e, quando necessário, avaliação histopatológica.

Giant cell tumor of the tendon sheath: a rare periungual location simulating myxoid cyst.

Giant cell tumor of the tendon sheath is a benign soft tissue tumor most frequent between the third and fifth decades of life. It can mimic and make differential diagnoses with several hand tumors. Definitive diagnosis and the treatment of choice are reached with complete resection and histopathological examination. Here we describe a case with clinical presentation similar to that of a myxoid cyst.

Giant cell tumor of the tendon sheath: a rare periungual location simulating myxoid cyst

ABSTRACT Giant cell tumor of the tendon sheath is a benign soft tissue tumor most frequent between the third and fifth decades of life. It can mimic and make differential diagnoses with several hand tumors. Definitive diagnosis and the treatment of choice are reached with complete resection and histopathological examination. Here we describe a case with clinical presentation similar to that of a myxoid cyst.