Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature

Clinical Sarcoma Research
Nicholas GiustiniArun S Singh

Abstract

Tenosynovial giant cell tumors (TGCTs) or giant cell tumors of tendon sheath are neoplasms that arise in the synovium. They can be categorized as nodular (localized) or diffuse type (D-TGCT). Historically, surgery has been the mainstay of therapy, but diffuse type disease recurs at a high rate and treatment often requires increasingly morbid procedures. Elucidation of the importance of the colony-stimulating factor (CSF1)/CSF1 receptor (CSF1R) pathway in the pathogenesis of this disease has created significant interest in targeting this pathway as a novel TGCT treatment approach. Pexidartinib, a selective tyrosine kinase inhibitor against CSF1R, showed an 83% disease control rate (52% with partial response and 31% with stable disease) in a recent phase 1 study of patients with TGCT. We present an illustrative example of a TGCT patient who would have required a morbid operation who derived considerable clinical benefit from pexidartinib treatment. Her tumor volume decreased by 48% after 4 months of treatment, and 55 months after starting treatment the patient exhibits continued disease stability with minimal clinical symptoms, and significant improvement in functional status. This case illustrates the effectiveness of systemic t...Continue Reading

References

Nov 26, 2002·Virchows Archiv : an International Journal of Pathology·M NilssonN Mandahl
Jan 13, 2006·Proceedings of the National Academy of Sciences of the United States of America·Robert B WestMatt van de Rijn
Oct 5, 2010·Seminars in Arthritis and Rheumatism·Sébastien OttavianiLaure Gossec
May 18, 2011·Current Opinion in Oncology·Vinod RaviValerae O Lewis
Jul 30, 2015·The New England Journal of Medicine·William D TapGideon Bollag
Apr 5, 2016·Clinical Therapeutics·Heather L GelhornWilliam D Tap
Jun 9, 2016·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Eric L StaalsEmanuela Palmerini
Jul 19, 2017·Journal for Immunotherapy of Cancer·Michael A CannarileDominik Rüttinger
Aug 9, 2017·Acta Orthopaedica·Monique J L MastboomUNKNOWN TGCT study group

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Citations

Oct 12, 2019·Drugs·Yvette N Lamb
Aug 14, 2019·Frontiers in Immunology·Nadine S JahchanMatthew F Krummel
Oct 23, 2019·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Carlos Murga-ZamalloaRyan A Wilcox
Mar 5, 2021·Radiology Case Reports·Stephanie D ZarateAna C Belzarena
Apr 4, 2021·Cancers·Tomohiro FujiwaraToshifumi Ozaki
Jul 19, 2021·Neuropeptides·Fatemeh TahmasebiIraj Ragerdi Kashani

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Methods Mentioned

BETA
biopsy
surgical resection
biopsies

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