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TGCT is a benign but destructive tumour of the joint lining, classed with soft-tissue sarcomas, in which a few cells carrying a CSF1 gene fusion recruit a crowd of normal immune cells that eat away at the joint. Surgery cures most localised cases, and for diffuse or recurrent disease two pills that block the CSF1 receptor, pexidartinib and vimseltinib, shrink tumours and restore joint function.
TGCT is a locally aggressive neoplasm of synovium, bursa and tendon sheath. Its biology is a landscape effect: a minority of neoplastic cells carry a translocation placing CSF1 under the COL6A3 promoter, overproducing colony-stimulating factor 1, which recruits CSF1R-expressing macrophages and osteoclast-like giant cells that make up most of the mass and cause pain, swelling, haemarthrosis and cartilage destruction. Localised (nodular) disease is cured by excision; diffuse disease (formerly pigmented villonodular synovitis) recurs after synovectomy in a large fraction of cases and can lead to joint replacement in young adults.
Because the tumour depends on CSF1 signalling, CSF1R inhibition is mechanistically exact. Pexidartinib (ENLIVEN, Lancet 2019) was the first FDA-approved systemic therapy for TGCT (August 2019), with objective responses and improved range of motion; it carries a boxed warning and REMS programme for serious and occasionally fatal cholestatic hepatotoxicity, which limited uptake and blocked EU approval. Vimseltinib, a switch-control CSF1R inhibitor, showed improved response and function versus placebo in MOTION (Lancet 2024) without the hepatotoxicity signal and was approved by the FDA in February 2025. Emactuzumab (anti-CSF1R antibody) is in the phase 3 TANGENT trial.
Open questions are treatment duration and rebound after stopping, the role of neoadjuvant CSF1R inhibition before surgery, and how to handle the many patients with disease that is symptomatic but not surgically threatening.
Localised disease is relatively common (tens of cases per million per year); the diffuse form that needs drugs is rare, at a few cases per million, mostly in adults aged 20 to 50.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Marginal excision; recurrence is uncommon and re-excision is curative in most cases.
Open or arthroscopic synovectomy by a sarcoma orthopaedic team; consider neoadjuvant CSF1R inhibition for large tumours (trial setting).
CSF1R inhibitor: vimseltinib (MOTION) or pexidartinib (ENLIVEN, with REMS hepatic monitoring); imatinib or nilotinib off label where neither is available.
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One cancer page, one trial page, one treatment page and one target page on OnCo cite this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing pages listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Tenosynovial giant cell tumour" OR ABSTRACT:"Tenosynovial giant cell tumour" OR TITLE:"TGCT" OR ABSTRACT:"TGCT" OR TITLE:"Pigmented villonodular synovitis" OR ABSTRACT:"Pigmented villonodular synovitis" OR TITLE:"PVNS" OR ABSTRACT:"PVNS" OR TITLE:"Giant cell tumour of the tendon sheath" OR ABSTRACT:"Giant cell tumour of the tendon sheath") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Tenosynovial giant cell tumour (TGCT), not a curated reading list.
West and colleagues show a CSF1-expressing neoplastic minority recruits the macrophage majority (PNAS).
Tap and colleagues, NEJM: high response rate in diffuse TGCT.
First systemic therapy approved for TGCT (August 2019), with a boxed hepatotoxicity warning.
Hepatotoxicity judged to outweigh benefit for a non-lethal disease.
Gelderblom and colleagues, Lancet.
FDA approval 14 February 2025 for symptomatic TGCT where surgery would worsen function.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Take with a meal and a large glass of water.
Take on an empty stomach (no food 2 hours before or 1 hour after): food raises exposure up to 82% and QT risk.
Known QT prolongation. Boxed warning for QT prolongation and sudden death: avoid QT-prolonging drugs; ECG at baseline, 7 days and after dose changes; correct potassium and magnesium.
Reduce dose in impairment per label.
A change on the heart's electrical trace, caused by some drugs blocking a potassium channel (hERG), that in rare cases sets off a dangerous rhythm. Drugs that do this need ECG checks and care with other medicines and low potassium or magnesium.
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