Tenosynovial Giant Cell Tumor

P=N/A, N=40, Not yet recruiting, Shanghai JiaoTong university Affiliated Sixth People‘s Hospital; Shanghai Jiao Tong University Affiliated Sixth People's Hospital

Systemic therapies targeting CSF1 or its receptor are valuable for relapsing or inoperable cases, especially D-TGCT, but their use is still limited by heavy side effects and unresponsive tumors. Overall, our understanding of the pathophysiology of these tumors, the efficacy and limitations of current therapeutic options, and the questions that remain unanswered open new avenues for research and provide opportunities to further improve patient care.

The CSF1-CSF1R and RANKL-RANK pathways are suitable targets for non-surgical interventions and inhibitors of these pathways are already being used for some entities in clinical practice. These inhibitors inhibit tumor growth and may induce bone formation, though pathologists should be aware when evaluating post treatment specimens that the neoplastic cells remain unaffected.

Whilst TSGCT typically arises at sites where synovium is present, its presence at the nasal dorsum introduced uncertainty regarding the tumour's pathogenesis. This case demonstrates the importance of integrating clinical, radiological, histopathological, and molecular findings in diagnosing rare presentations of soft tissue tumours and expands the anatomical locations at which TSGCT may arise.

This review summarizes current knowledge of the mechanisms underlying tumorigenesis in TGCT, providing an overview of the key drivers and corresponding therapeutic targets. Further research into the specific mechanisms of TGCT and further development of diagnostics and treatments are warranted.

P3, N=90, Active, not recruiting, Abbisko Therapeutics Co, Ltd | Trial primary completion date: May 2026 --> Sep 2024

The findings suggest that targeting ADGRE5 and NF-κB could offer new therapeutic strategies for controlling disease progression and reducing joint destruction in PVNS patients. Further research is needed to elucidate this disease's specific regulatory mechanisms and cell types.

To the best of our knowledge, this is the first example of genetically confirmed TGCT in the nasal cavity. Our study broadens the anatomical spectrum of TGCT and highlights the inclusion of TGCT in the differential diagnosis of nasal cavity lesions.

P=N/A, N=100, Recruiting, Deciphera Pharmaceuticals, LLC | Not yet recruiting --> Recruiting

P=N/A, N=30, Recruiting, Daiichi Sankyo | Trial completion date: Mar 2036 --> Jun 2036 | Trial primary completion date: Jun 2025 --> Jun 2036

P=N/A, N=100, Not yet recruiting, Deciphera Pharmaceuticals, LLC

P1, N=54, Recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2025 --> Dec 2028 | Trial primary completion date: Dec 2025 --> Dec 2026