PMID- 26988130 OWN - NLM STAT- MEDLINE DCOM- 20170310 LR - 20220330 IS - 1744-7658 (Electronic) IS - 1354-3784 (Linking) VI - 25 IP - 6 DP - 2016 Jun TI - Investigational therapies for Ewing sarcoma: a search without a clear finding. PG - 679-86 LID - 10.1517/13543784.2016.1168398 [doi] AB - INTRODUCTION: Ewing sarcoma family tumors (ESFT) are a group of aggressive diseases, characterized histologically by small, round, blue cells and genetically by translocation involving EWS and ETS partner genes. The current treatment of localized Ewing sarcoma (ES) requires a multi-disciplinary approach, including multidrug chemotherapy, administrated before and after local treatment, surgery and radiation therapy. Unfortunately, the cure rate of metastatic or refractory/recurrent disease is still very poor. AREAS COVERED: In this review, the authors summarize the new types of therapy and strategies aimed to improve the prognosis or cure ES. Herein, the authors discuss several preclinical and phase I-II studies with new-targeted therapies. The most studied therapies are insulin-like growth factor receptor (IGF1R) inhibitors but have limited efficacy. Other strategies include Mammalian Target of Rapamycin (mTOR) Inhibition, poly ADP ribose polymerase (PARP) inhibition, vascular endothelial growth factor (VEGF) inhibition, tyrosine kinase inhibitors and telomerase inhibitors, all with limited effectiveness. EXPERT OPINION: Future treatment strategies should combine one or more targeted therapies with conventional chemotherapy. Some combined modality treatments are under clinical study. However, treatment breakthroughs are still needed to improve the relatively poor prognosis of recurrent/metastatic ESFT. FAU - Vornicova, Olga AU - Vornicova O AD - a Division of Oncology, Rambam Health Care Campus and Faculty of Medicine , Technion-Israel Institute of Technology , Haifa , Israel. FAU - Bar-Sela, Gil AU - Bar-Sela G AD - a Division of Oncology, Rambam Health Care Campus and Faculty of Medicine , Technion-Israel Institute of Technology , Haifa , Israel. LA - eng PT - Journal Article PT - Review DEP - 20160407 PL - England TA - Expert Opin Investig Drugs JT - Expert opinion on investigational drugs JID - 9434197 RN - 0 (Antineoplastic Agents) SB - IM MH - Animals MH - Antineoplastic Agents/pharmacology/therapeutic use MH - Bone Neoplasms/*drug therapy/pathology MH - Combined Modality Therapy MH - Humans MH - Molecular Targeted Therapy MH - Neoplasm Recurrence, Local MH - Prognosis MH - Sarcoma, Ewing/pathology/*therapy MH - Therapies, Investigational/*methods OTO - NOTNLM OT - Ewing sarcoma family tumors OT - IGF1R OT - PARP OT - mTOR OT - review OT - target therapy EDAT- 2016/03/19 06:00 MHDA- 2017/03/11 06:00 CRDT- 2016/03/19 06:00 PHST- 2016/03/19 06:00 [entrez] PHST- 2016/03/19 06:00 [pubmed] PHST- 2017/03/11 06:00 [medline] AID - 10.1517/13543784.2016.1168398 [doi] PST - ppublish SO - Expert Opin Investig Drugs. 2016 Jun;25(6):679-86. doi: 10.1517/13543784.2016.1168398. Epub 2016 Apr 7.