Tenosynovial giant cell tumor

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 non-malignant tumor defined histologically as inclusions of “osteoclast-like” multinucleated giant cells, hemosiderin, and macrophages.[1] This histology can present one of 2 clinically distinct ways. Localized/nodular TGCT (L-TGCT), sometimes referred to as “giant cell tumor of the tendon sheath”;[2]: 100 is a common tumor that presents as a slow-growing, encapsulated, localized and limited bump, most frequently in the fingers. [3] Diffuse TGCT (D-TGCT) — also called pigmented villonodular synovitis (PVNS)— is a rare tumor that presents as a proliferative, destructive, intra-articular lesion, most commonly in the knee.[2]: 102 [4] D-TGCT tumors often develop from the lining of joints (also known as synovial tissue).[5][2][2]: 100[6][6]: 245. Common symptoms of D-TGCT include swelling, pain, stiffness and reduced mobility in the affected joint or limb.[2]: 102

  1. ^ Choi WS, Lee SK, Kim JY, Kim Y (2024-01-17). "Diffuse-Type Tenosynovial Giant Cell Tumor: What Are the Important Findings on the Initial and Follow-Up MRI?". Cancers (Basel). 16 (2): 402. doi:10.3390/cancers16020402. PMC 10814250. PMID 38254890.
  2. ^ 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.
  3. ^ Gouin F, Noailles T (2017). "Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis)". Orthopaedics & Traumatology, Surgery & Research. 103 (1S): S91 – S97. doi:10.1016/j.otsr.2016.11.002. PMID 28057477. Retrieved 2024-12-18.
  4. ^ Choi WS, Lee SK, Kim JY, Kim Y (2024). "Diffuse-Type Tenosynovial Giant Cell Tumor: What Are the Important Findings on the Initial and Follow-Up MRI?". Cancers. 16 (2): 402. doi:10.3390/cancers16020402. PMC 10814250. PMID 38254890.
  5. ^ 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.
  6. ^ 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.