Tenosynovial giant cell tumor

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Section editor
Elizabeth J. Davis, MD
Vanderbilt University
Nashville, TN, USA

Are you looking for a regimen but can't find it here? For placebo or observational studies in this condition, please visit this page. If you still can't find it, please let us know so we can add it!
Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens.

1 regimens on this page
1 variants on this page


Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.


All lines of therapy

Pexidartinib monotherapy


Study Dates of enrollment Evidence Comparator Comparative Efficacy
Tap et al. 2019 (ENLIVEN) 2015-05 to 2016-09 Phase 3 (E-RT-esc) Placebo1 Superior ORR (primary endpoint)

1Patents assigned to placebo were offered open-label pexidartinib after week 24.

Targeted therapy

  • Pexidartinib (Turalio) as follows:
    • Cycle 1: 400 mg PO every morning and 600 mg PO every evening
    • Cycle 2 onwards: 400 mg PO twice per day

14-day cycles


  1. ENLIVEN: Tap WD, Gelderblom H, Palmerini E, Desai J, Bauer S, Blay JY, Alcindor T, Ganjoo K, Martín-Broto J, Ryan CW, Thomas DM, Peterfy C, Healey JH, van de Sande M, Gelhorn HL, Shuster DE, Wang Q, Yver A, Hsu HH, Lin PS, Tong-Starksen S, Stacchiotti S, Wagner AJ; ENLIVEN investigators. Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial. Lancet. 2019 Aug 10;394(10197):478-487. Epub 2019 Jun 19. link to original article link to PMC article contains dosing details in manuscript PubMed NCT02371369