Tenosynovial giant cell tumor
| 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 non-malignant tumor defined histologically as inclusions of “osteoclast-like” multinucleated giant cells, hemosiderin, and macrophages. 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”;: 100 is a common tumor that presents as a slow-growing, encapsulated, localized and limited bump, most frequently in the fingers. 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.: 102 D-TGCT tumors often develop from the lining of joints (also known as synovial tissue).: 100 : 245 . Common symptoms of D-TGCT include swelling, pain, stiffness and reduced mobility in the affected joint or limb.: 102