[Recurrent or refractory Osteosarcoma and Ewing sarcoma-French guidelines from the FSG/NETSARC and GroupOs groups].

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Abstract

[Indexed for MEDLINE] Conflict of interest statement: Déclaration de liens d’intérêts les auteurs déclarent ne pas avoir de liens d’intérêts. 20. Paediatr Drugs. 2015 Aug;17(4):257-71. doi: 10.1007/s40272-015-0134-4. Bone Sarcomas in Pediatrics: Progress in Our Understanding of Tumor Biology and Implications for Therapy. Rivera-Valentin RK(1), Zhu L, Hughes DP. Author information: (1)Department of Pediatrics-Research, The Children's Cancer Hospital at MD Anderson Cancer Center, Unit 853, MOD 1.021d, 1515 Holcombe Blvd, Houston, TX, 77030, USA. The pediatric bone sarcomas osteosarcoma and Ewing sarcoma represent a tremendous challenge for the clinician. Though less common than acute lymphoblastic leukemia or brain tumors, these aggressive cancers account for a disproportionate amount of the cancer morbidity and mortality in children, and have seen few advances in survival in the past decade, despite many large, complicated, and expensive trials of various chemotherapy combinations. To improve the outcomes of children with bone sarcomas, a better understanding of the biology of these cancers is needed, together with informed use of targeted therapies that exploit the unique biology of each disease. Here we summarize the current state of knowledge regarding the contribution of receptor tyrosine kinases, intracellular signaling pathways, bone biology and physiology, the immune system, and the tumor microenvironment in promoting and maintaining the malignant phenotype. These observations are coupled with a review of the therapies that target each of these mechanisms, focusing on recent or ongoing clinical trials if such information is available. It is our hope that, by better understanding the biology of osteosarcoma and Ewing sarcoma, rational combination therapies can be designed and systematically tested, leading to improved outcomes for a group of children who desperately need them. DOI: 10.1007/s40272-015-0134-4 PMCID: PMC4516866