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|Ref Type||Journal Article|
|Authors||Lopez-Pousa A, Martin Broto J, Garrido T, Vazquez J|
|Title||Giant cell tumour of bone: new treatments in development.|
|Journal||Clinical & translational oncology : official publication of the Federation of|
|Abstract Text||Giant cell tumour of bone (GCTB) is a benign osteolytic tumour with three main cellular components: multinucleated osteoclast-like giant cells, mononuclear spindle-like stromal cells (the main neoplastic components) and mononuclear cells of the monocyte/macrophage lineage. The giant cells overexpress a key mediator in osteoclastogenesis: the RANK receptor, which is stimulated in turn by the cytokine RANKL, which is secreted by the stromal cells. The RANK/RANKL interaction is predominantly responsible for the extensive bone resorption by the tumour. Historically, standard treatment was substantial surgical resection, with or without adjuvant therapy, with recurrence rates of 20-56 %. Studies with denosumab, a monoclonal antibody that specifically binds to RANKL, resulted in dramatic treatment responses, which led to its approval by the United States Food and Drugs Administration (US FDA). Recent advances in the understanding of GCTB pathogenesis are essential to develop new treatments for this locally destructive primary bone tumour.|
|Molecular Profile||Treatment Approach|
|Gene Name||Source||Synonyms||Protein Domains||Gene Description||Gene Role|
|Therapy Name||Drugs||Efficacy Evidence||Clinical Trials|
|Drug Name||Trade Name||Synonyms||Drug Classes||Drug Description|
|Gene||Variant||Impact||Protein Effect||Variant Description||Associated with drug Resistance|
|Molecular Profile||Indication/Tumor Type||Response Type||Therapy Name||Approval Status||Evidence Type||Efficacy Evidence||References|
|Unknown unknown||bone giant cell tumor||not applicable||Denosumab||FDA approved||Actionable||In a Phase II clinical trial that supported FDA approval, Xgeva (denosumab) treatment resulted in an overall objective response rate of 25% (47/187) in patients with giant cell bone tumor, with a median duration of response of 20 months among patients demonstrating response (PMID: 25617146).||detail... 25617146|