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Tenosynovial giant cell tumor information


Tenosynovial giant cell tumor
Other namesLocalized: Localized pigmented villonodular synovitis (L-PVNS), Giant cell tumor of the tendon sheath (GCT-TS), Nodular tenosynovitis, Localized nodular tenosynovitis, and L-TGCT
Diffuse: Pigmented villonodular synovitis (PVNS), Conventional PVNS, and D-TGCT
Micrograph of diffuse TGCT, also known as pigmented villonodular synovitis. H&E stain.
SpecialtyOncology
SymptomsSwelling, pain, stiffness, sensitivity, and/or limited range of motion
ComplicationsOsteoarthritis
Usual onsetMost patients are diagnosed between the age of 30-50.
TypesDiffuse and localized
Diagnostic methodMRI, biopsy, surgery
Differential diagnosisFibromas, Baker’s cyst, tophaceous gout, synovial sarcoma, hemangioma, synovial chondromatosis, hemorrhagic synovitis
TreatmentSurgery, CSF1R inhibitors
MedicationImatinib, Pexidartinib, vimseltinib

Tenosynovial giant cell tumor (TGCT) is a group of rare, typically non-malignant tumors of the joints. TGCT tumors often develop from the lining of joints (also known as synovial tissue).[1][2][2]: 100[3][3]: 245.

Common symptoms of TGCT include swelling, pain, stiffness and reduced mobility in the affected joint or limb.[2]: 102 This group of tumors can be divided into different subsets according to their site, growth pattern, and prognosis.[4][4]: 361 Localized/nodular TGCT is sometimes referred to as giant cell tumor of the tendon sheath;[2]: 100 diffuse TGCT is also called pigmented villonodular synovitis (PVNS).[2]: 102These two distinct subtypes determined by radiographic appearance.[5] Localized TGCT is defined as a well circumscribed tumor while diffuse TGCT exhibits a locally aggressive and infiltrative behavior.[6]

  1. ^ Lucas, David R. (2012). "Tenosynovial Giant Cell Tumor: Case Report and Review". Archives of Pathology & Laboratory Medicine. 136 (8): 901–906. doi:10.5858/arpa.2012-0165-CR. PMID 22849738.
  2. ^ a b c d e Fletcher, C. D.M.; Bridge, J.A.; Hogendoorn, P.; Mertens, F. (2013). WHO Classification of Tumours of Soft Tissue and Bone. Fourth Edition. World Health Organization. ISBN 9789283224341. Archived from the original on July 19, 2016.
  3. ^ a b Rateb, Kochbati; Hassen, Ben Ghozlen; Leila, Abid; Faten, Farah; Med Samir, Daghfous (2017). "Giant cell tumor of soft tissues: A case report of extra-articular diffuse-type giant cell tumor of the quadriceps". International Journal of Surgery Case Reports. 31: 245–249. doi:10.1016/j.ijscr.2016.12.019. PMC 5310176. PMID 28199932.
  4. ^ a b Ravi, Vinod; Wang, Wei-Lien; Lewis, Valerae O. (2011). "Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis". Current Opinion in Oncology. 23 (4): 361–366. doi:10.1097/CCO.0b013e328347e1e3. PMID 21577109. S2CID 1608847.
  5. ^ Stacchiotti, Silvia; Dürr, Hans Roland; Schaefer, Inga-Marie; Woertler, Klaus; Haas, Rick; Trama, Annalisa; Caraceni, Augusto; Bajpai, Jyoti; Baldi, Giacomo Giulio; Bernthal, Nicholas; Blay, Jean-Yves; Boye, Kjetil; Broto, Javier-Martin; Chen, Wei-Wu Tom; Dei Tos, Paolo Angelo (January 2023). "Best clinical management of tenosynovial giant cell tumour (TGCT): A consensus paper from the community of experts". Cancer Treatment Reviews. 112: 102491. doi:10.1016/j.ctrv.2022.102491. hdl:10067/1923310151162165141. PMID 36502615.
  6. ^ Palmerini, Emanuela; Staals, Eric L.; Maki, Robert G.; Pengo, Stefano; Cioffi, Angela; Gambarotti, Marco; Picci, Piero; Daolio, Primo Andrea; Parafioriti, Antonina; Morris, Carol; Antonescu, Cristina R.; Gronchi, Alessandro; Casali, Paolo Giovanni; Donati, Davide M.; Ferrari, Stefano (January 2015). "Tenosynovial giant cell tumour/pigmented villonodular synovitis: Outcome of 294 patients before the era of kinase inhibitors". European Journal of Cancer. 51 (2): 210–217. doi:10.1016/j.ejca.2014.11.001. PMID 25465190.

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