Giant cell tumour of bone: new treatments in development

A. López-Pousa ^[2] ; J. Martín Broto ^[1] ; T. Garrido ^[3] ; J. Vázquez ^[3]
1. [1] Hospital Universitario Son Espases
  
  Hospital Universitario Son Espases
  
  Palma de Mallorca, España
2. [2] Hospital de la Santa Creu i Sant Pau
  
  Hospital de la Santa Creu i Sant Pau
  
  Barcelona, España
3. [3] Amgen S.A., Barcelona
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Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 17, Nº. 6, 2015, págs. 419-430
Idioma: inglés
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Resumen
- 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.