Advances in treatment for tenosynovial giant cell tumors

Research output: Contribution to journalReview articlepeer-review


Introduction: Tenosynovial giant cell tumor (TGCT) is a benign clonal neoplastic proliferation arising from the synovium often causing pain, swelling, joint stiffness, and reduced quality of life. The optimal treatment strategy in patients with diffuse-type TGCT (dt-TGCT) is unclear. The purpose of this review is to describe recent advances in the knowledge of TGCT pathogenesis and potential therapeutic implications. Areas covered: Current treatment options for TGCT are discussed, including surgery and radiotherapy. Recent evidence that TGCT cells overexpress colony-stimulating factor 1 (CSF1), resulting in recruitment of CSF1 receptor (CSF1R) positive tumor-associated macrophage and contributing to tumor growth, has created new opportunities for systemic treatments of dt-TGCT. Results of clinical trials with CSF1R inhibitors are now available. These inhibitors include small molecules such as imatinib, nilotinib, pexidartinib, and monoclonal antibodies like emactuzumab, MCS110, and cabiralzumab. Expert opinion: TGCT impairs patients’ quality of life significantly. The confirmation that the pathogenetic loop of TGCT can be inhibited through targeted agents could potentially change the therapeutic armamentarium for this condition.

Original languageEnglish
Pages (from-to)753-757
Number of pages5
JournalExpert Opinion on Orphan Drugs
Issue number12
Publication statusPublished - Dec 2 2018


  • colony-stimulating factor 1 (CSF1)
  • CSF1R
  • Pigmented villonodular synovitis
  • PVNS
  • surgery
  • tenosynovial giant cell tumor
  • TGCT
  • treatment

ASJC Scopus subject areas

  • Pharmacology, Toxicology and Pharmaceutics (miscellaneous)
  • Health Policy
  • Pharmacology (medical)


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