| Tenosynovial giant cell tumor | |
|---|---|
| Other names | Localized: 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. | |
| Specialty | Oncology |
| Symptoms | Swelling, pain, stiffness, sensitivity, and/or limited range of motion |
| Complications | Osteoarthritis |
| Usual onset | Most patients are diagnosed between the age of 30-50. |
| Types | Diffuse and localized |
| Diagnostic method | MRI, biopsy, surgery |
| Differential diagnosis | Fibromas, Baker’s cyst, tophaceous gout, synovial sarcoma, hemangioma, synovial chondromatosis, hemorrhagic synovitis |
| Treatment | Surgery, CSF1R inhibitors |
| Medication | Imatinib, 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]: 102 These 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]
- ^ Lucas DR (August 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.
- ^ a b c d e Fletcher CD, Bridge J, Hogendoorn P, Mertens F (2013). WHO Classification of Tumours of Soft Tissue and Bone (Fourth ed.). World Health Organization. ISBN 9789283224341. Archived from the original on July 19, 2016.
- ^ a b Rateb K, Hassen BG, Leila A, Faten F, Med Samir D (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.
- ^ a b Ravi V, Wang WL, Lewis VO (July 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.
- ^ Stacchiotti S, Dürr HR, Schaefer IM, Woertler K, Haas R, Trama A, et al. (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.
- ^ Palmerini E, Staals EL, Maki RG, Pengo S, Cioffi A, Gambarotti M, et al. (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.