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Pexidartinib and CSF1R inhibitors As Treatment for Tenosynovial Giant Cell Tumors

Published Online: December 23rd 2020 Oncology & Hematology Review (US). 2020;16(2):Online ahead of journal publication
Authors: Caroline J Granger, Ty Subhawong, Gina D’Amato, Emily Jonczak, Erika Garbrecht, Sheila A Conway, Jonathan C Trent
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Tenosynovial Giant Cell Tumor (TGCT), previously referred to as giant cell tumor of the tendon sheath or pigmented villonodular synovitis (PVNS), is a largely benign, rare, proliferative lesion arising from the synovial lining of joints, bursae, and tendon sheaths. TGCT has been classified into two clinically distinct yet genetically identical localized (TGCT-L), which is typically cured with surgery alone, or diffuse types (TGCT-D), which is much more difficult to manage. A translocation involving the short arm of chromosome 1p11-13, with resultant hyperexpression of macrophage colony-stimulating factor 1 (CSF1), has since been shown to contribute in large part to the development of TGCT. Excessive resultant CSF1 secretion attracts monocytes and macrophages due to their expression of the CSF1 receptor (CSF1R).CSF1R inhibitors have been used with promise to improve patient outcomes in TGCT-D. Their role is increasing but is still largely undefined. This review serves to discuss TGCT’s epidemiology, molecular biology, clinical behavior, and the current treatment modalities being effectively utilized for patients with TGCT with a specific focus on CSF1R inhibitors, namely, the newly FDA-approved (in the United States) pexidartinib.


Tenosynovial giant cell tumor, colony-stimulating factor 1, CSF1R inhibitors, pexidartinib


**This manuscript has been accepted for publication, but not yet copyedited or typeset, and may be subject to minor changes during the production process**

Article Information:

None of the authors have any financial or non-financial relationships or activities to declare in relation to this article.
Research was funded in part through the NIH/NCI Cancer Center Support Grant P30CA240139 (JCT).


Compliance With Ethics

This article involves a review of the literature and did not involve any studies with human or animal subjects performed by any of the authors.

Review Process

Double-blind peer review.


The named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship of this manuscript, take responsibility for the integrity of the work as a whole, and have given final approval for the version to be published.


Jonathan Trent, MD, PhD, 1475 NW 12th Ave Floor 2, Miami, FL 33136, USA.
E: jtrent@miami.edu
Twitter: @JTrentMDPhD



No funding was received in the publication of this article.

Open Access

This article is freely accessible at touchONCOLOGY.com  © Touch Medical Media 2020.



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