Bone Tumor Environment as a Potential Therapeutic Target in Ewing Sarcoma.

摘要: Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, an aggressive, rapidly fatal malignancy that mainly develops not only in osseous sites (85%) but also extra-skeletal soft tissue. It spreads naturally to lungs, bones, and marrow poor prognosis two latter cases. Bone lesions from primary or secondary (metastases) tumors are characterized by extensive remodeling, more often due osteolysis. Osteoclast activation subsequent resorption responsible for features of tumors, including pain, vertebral collapse, spinal cord compression. Based on "vicious cycle" concept tumor cells resorbing cells, drugs, which target osteoclasts, may be promising agents as adjuvant setting treating sarcoma. There increasing evidence cellular molecular protagonists present microenvironment play a part establishing favorable "niche" initiation progression. The purpose this review discuss potential therapeutic value drugs targeting first will focus function osteoclasts means bisphosphonates blocking pro-resorbing cytokine receptor activator NF-kappa B ligand. Second, role peculiar hypoxic discussed context resistance chemotherapy, escape immune system, neo-angiogenesis. Therapeutic interventions based these specificities could then proposed