PMID- 23760780 OWN - NLM STAT- MEDLINE DCOM- 20140131 LR - 20211203 IS - 1179-2019 (Electronic) IS - 1174-5878 (Linking) VI - 15 IP - 6 DP - 2013 Dec TI - Optimal management of Ewing sarcoma family of tumors: recent developments in systemic therapy. PG - 473-92 LID - 10.1007/s40272-013-0037-1 [doi] AB - The Ewing sarcoma family of tumors (ESFT) is defined by cell surface expression of CD99 and a translocation involving EWS and an ETS partner. Cytotoxic chemotherapy remains the benchmark of first- and second-line therapy, and although the majority of patients with localized disease are cured, almost one third of patients relapse or progress from their disease. Moreover, cure remains elusive in most patients who present with distant metastases. In recent years, the ESFT literature has been dominated by reports of attempts at modulating the insulin-like growth factor (IGF) receptor (IGFR). Unfortunately, three phase II studies examining inhibiting antibodies to IGFR-1 published disappointing results. Whether these results were due to failure to modulate the pathway or other limitations in study design and/or patient selection remain unclear. Other novel strategies currently being investigated in ESFT include tyrosine kinase, mammalian target of rapamycin (mTOR), and poly(ADP-ribose) polymerase (PARP) inhibitors. FAU - Owens, Cormac AU - Owens C AD - The Division of Hematology/Oncology, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, M5G 1N6, Canada, cormacowens@yahoo.com. FAU - Abbott, Lesleigh S AU - Abbott LS FAU - Gupta, Abha A AU - Gupta AA LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Paediatr Drugs JT - Paediatric drugs JID - 100883685 RN - 0 (Poly(ADP-ribose) Polymerase Inhibitors) RN - 0 (Protein Kinase Inhibitors) RN - 67763-96-6 (Insulin-Like Growth Factor I) RN - EC 2.7.1.1 (MTOR protein, human) RN - EC 2.7.10.1 (Protein-Tyrosine Kinases) RN - EC 2.7.11.1 (TOR Serine-Threonine Kinases) SB - IM MH - Animals MH - Bone Neoplasms/*drug therapy/genetics/surgery MH - Chemotherapy, Adjuvant MH - Humans MH - Insulin-Like Growth Factor I/metabolism MH - Metabolic Networks and Pathways MH - Neoplasm Recurrence, Local/drug therapy MH - Poly(ADP-ribose) Polymerase Inhibitors MH - Protein Kinase Inhibitors/*therapeutic use MH - Protein-Tyrosine Kinases/antagonists & inhibitors MH - Sarcoma, Ewing/*drug therapy/genetics/surgery MH - TOR Serine-Threonine Kinases/antagonists & inhibitors MH - Translocation, Genetic EDAT- 2013/06/14 06:00 MHDA- 2014/02/01 06:00 CRDT- 2013/06/14 06:00 PHST- 2013/06/14 06:00 [entrez] PHST- 2013/06/14 06:00 [pubmed] PHST- 2014/02/01 06:00 [medline] AID - 10.1007/s40272-013-0037-1 [doi] PST - ppublish SO - Paediatr Drugs. 2013 Dec;15(6):473-92. doi: 10.1007/s40272-013-0037-1.