Tenosynovial giant cell tumor-diffuse type, treated with a novel colony-stimulating factor inhibitor, pexidartinib: initial experience with MRI findings in three patients

Tenosynovial giant cell tumor-diffuse type (diffuse TSGCT) is a benign but locally aggressive proliferative disorder of the synovium. Treatment is usually surgical, although in cases of extensive disease complete synovectomy is not possible and local recurrence rates are high. Pexidartinib (trade na...

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Bibliographic Details
Published in:Skeletal radiology 2022-05, Vol.51 (5), p.1085-1091
Main Authors: Helming, Andrew, Hansford, Barry, Beckett, Brooke
Format: Article
Language:English
Subjects:
Care and treatment
Case Report
Colonies
Colony-stimulating factor
Drug approval
Health services
Hepatotoxicity
Imaging
Inhibitors
Macrophage colony-stimulating factor
Magnetic resonance imaging
Medical imaging
Medical records
Medical research
Medicine
Medicine & Public Health
Medicine, Experimental
Morbidity
Nuclear Medicine
Orthopedics
Pathology
Patients
Radiology
Risk assessment
Synovium
Tumors
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Summary:Tenosynovial giant cell tumor-diffuse type (diffuse TSGCT) is a benign but locally aggressive proliferative disorder of the synovium. Treatment is usually surgical, although in cases of extensive disease complete synovectomy is not possible and local recurrence rates are high. Pexidartinib (trade name Turalio®), a colony-stimulating factor-1 (CSF-1) inhibitor, was shown in a recent phase III trial to effectively treat diffuse TSGCT and is FDA approved for the treatment of adult patients with symptomatic diffuse TSGCT associated with severe morbidity or functional limitations and not amenable to improvement with surgery. Pexidartinib is available only through a restricted program under a risk evaluation and mitigation strategy (REMS) because of the risk of hepatotoxicity. Magnetic resonance imaging (MRI) is the preferred imaging modality for the diagnosis and surveillance of TSGCT. Here we present three patients with diffuse TSGCT of the knee who underwent multiple MRIs over several years while on pexidartinib. We describe the disease burden and signal characteristics on MRI and correlate with the response reported in the patients’ medical records. Given that the use of pexidartinib and other CSF inhibitors is likely to increase, musculoskeletal radiologists should be aware of this novel non-operative treatment and the MRI appearance of diffuse TSGCT during therapy.
ISSN:0364-2348
1432-2161
DOI:10.1007/s00256-021-03924-3