Identifier to cite or link to this item: http://hdl.handle.net/20.500.13003/12478
Giant cell tumour of bone: new treatments in development
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ISSN: 1699-048X
eISSN: 1699-3055
WOS ID: 000355340900001
Scopus EID: 2-s2.0-84930088116
PMID: 25617146
Embase PUI: L602142260
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2015-06Document type
research articleCitation
Lopez-Pousa A, Martin Broto J, Garrido T, Vazquez J. Giant cell tumour of bone: new treatments in development. Clin Transl Oncol. 2015 Jun;17(6):419-30. Epub 2015 Jan 24.Abstract
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.