Origin and Therapies of Osteosarcoma

Abstract

Osteosarcoma (OS) is the most frequent primary bone tumor, mainly affecting children and young adults. Despite therapeutic advances, the 5-year survival rate is 70% but drastically decreases to 20-30% for poor responders to therapies or for patients with metastasis. No real evolution of the survival rates has been observed for four decades, explained by poor knowledge of the origin, difficulties related to diagnosis and the lack of targeted therapies for this pediatric tumor. This review will describe a non-exhaustive overview of osteosarcoma disease from a clinical and biological point of view, describing the origin, diagnosis and therapies.

Keywords: origin; osteosarcoma; therapy.

Figure 1

The vicious cycle in osteosarcoma: the cancer cell secretes growth factors that activate osteoblasts. These, together with the tumor cell, promote osteoclastic differentiation and exaggerated resorption of the bone matrix. This resorption leads to the release into the microenvironment of factors involved in tumor survival and proliferation. PTH/rP: parathyroid hormone-related protein, IGF: insulin-like growth factor, FGF: fibroblast growth factor, VEGF: vascular endothelial growth factor, IL-(1, 6, 11): interleukin-(1, 6, 11, RANKL: receptor activator of NF-kB ligand, c-fms: colony stimulating factor 1 receptor, TGF-ß: transforming growth factor ß; BMP: bone morphogenetic protein, PDGF: platelet-derived growth factor, M-CSF: macrophage colony-stimulating factor, Ca²⁺: calcium ions.