The first 60 days: Tenosynovial giant cell tumour (TGCT)
TGCT is a benign but destructive tumour of the joint lining 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. Below, week by week, is what OnCo's record of Tenosynovial giant cell tumour (TGCT) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- SurgeonNamed in the standard of care for: Localised TGCT, Diffuse TGCT, resectable.
- Medical oncologistNamed in the standard of care for: Diffuse TGCT where surgery would cause severe morbidity or after recurrence.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
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).
- 3.Diffuse TGCT where surgery would cause severe morbidity or after recurrenceNCCN category Category 2A, NCCN Soft Tissue Sarcoma
CSF1R inhibitor: vimseltinib (MOTION) or pexidartinib (ENLIVEN, with REMS hepatic monitoring); imatinib or nilotinib off label where neither is available.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example CSF1 rearrangementin neoplastic cells, CSF1R-positive macrophage-rich infiltrate, MRI pattern, Liver function tests before and during pexidartinib), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include LocalisedTGCT, Diffuse TGCT, Malignant TGCT.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised TGCT
- For my situation (localised tgct), which of the standard options do you recommend and why?Guideline options include: Marginal excision; recurrence is uncommon and re-excision is curative in most cases.
Diffuse TGCT, resectable
- For my situation (diffuse tgct, resectable), which of the standard options do you recommend and why?Guideline options include: Open or arthroscopic synovectomy by a sarcoma orthopaedic team; consider neoadjuvant CSF1R inhibition for large tumours (trial setting).
Diffuse TGCT where surgery would cause severe morbidity or after recurrence
- For my situation (diffuse tgct where surgery would cause severe morbidity or after recurrence), which of the standard options do you recommend and why?Guideline options include: CSF1R inhibitor: vimseltinib (MOTION) or pexidartinib (ENLIVEN, with REMS hepatic monitoring); imatinib or nilotinib off label where neither is available.
- Am I a candidate for Vimseltinib, Pexidartinib, Imatinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of MOTION apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of Vimseltinib, MOTION, Emactuzumab?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Duration of CSF1R therapy and rebound after stopping: extension cohorts and intermittent schedules are being studied”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Hepatotoxicity of pexidartinib: newer agents (vimseltinib, emactuzumab) are designed to avoid it”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- A Study of the Efficacy and Safety of Pexidartinib in Adult Subjects With TGCTPhase 3 · active · NCT04488822Multicenter, Single Arm Study of the Efficacy and Safety of Pexidartinib in Adult Subjects With Tenosynovial Giant Cell Tumor
- Study of Emactuzumab for Tenosynovial Giant Cell Tumor (TGCT)Phase 3 · active · NCT05417789A Phase III, Multicentre, Randomised, Double-Blind Study to Assess the Safety and Efficacy of Emactuzumab vs. Placebo in Subjects With Tenosynovial Giant Cell Tumour
- Study of Pimicotinib (ABSK021) for Tenosynovial Giant Cell Tumor (MANEUVER)Phase 3 · active · NCT05804045A Phase 3, Randomized, Double-blind, Placebo-Controlled, Multicenter Study of ABSK021 to Assess the Efficacy and Safety in Patients With Tenosynovial Giant Cell Tumor
- Study of Pimicotinib in Japanese Participants With Tenosynovial Giant Cell Tumor (TGCT) (J-MANEUVER)Phase 2 · recruiting · NCT07499362Phase 2, Single-arm Study to Investigate the Tolerability, Pharmacokinetics, Efficacy, and Safety of Pimicotinib in Japanese Participants With Tenosynovial Giant Cell Tumor
- Study of Vimseltinib (DCC-3014) in Patients With Advanced Tumors and Tenosynovial Giant Cell TumorPhase 1/2 · active · NCT03069469A Multicenter Phase 1/2, Open-Label Study of DCC-3014 to Assess the Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics in Patients With Advanced Tumors and Tenosynovial Giant Cell Tumor
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Tenosynovial giant cell tumour (TGCT): the full pageTGCT is a benign but destructive tumour of the joint lining 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Every term links to the glossary.