PMID- 18345719 OWN - NLM STAT- MEDLINE DCOM- 20080808 LR - 20211203 IS - 1174-5878 (Print) IS - 1174-5878 (Linking) VI - 10 IP - 2 DP - 2008 TI - Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma. PG - 93-105 AB - The Ewing sarcoma family of tumors (ESFT) is one of the most common groups of malignancies arising in children, adolescents, and young adults up to approximately 25 years of age. It comprises Ewing sarcoma arising from bone and extraosseous Ewing sarcoma arising from soft tissues (which includes peripheral neuroectodermal tumors and Askin tumor arising from the chest wall). Ewing sarcoma is treated successfully in many cases by a combination of chemotherapy, surgery, and radiotherapy. A number of prognostic factors have been identified that can be used to stratify patients according to the risk of relapse, allowing optimization of treatment. These can be categorized as tumor-related factors (presence of metastases, tumor site, volume, lactic dehydrogenase level, chromosomal translocation type, presence of fusion transcripts in blood and bone marrow), treatment-related factors (local therapy, histologic response to chemotherapy, radiologic response to chemotherapy, chemotherapy regimen), and patient-related factors (gender, age). Newer chemotherapeutic agents are currently being investigated, and there is now increasing interest in the identification of molecular targets in ESFT that could be exploited therapeutically, which include the mammalian target of rapamycin (mTOR) and insulin growth factor-1 (IGF-1) receptor pathways. FAU - Seddon, Beatrice M AU - Seddon BM AD - London Bone and Soft Tissue Tumor Service, University College Hospital, London, UK. beatrice.seddon@uclh.nhs.uk FAU - Whelan, Jeremy S AU - Whelan JS LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Paediatr Drugs JT - Paediatric drugs JID - 100883685 RN - EC 2.7.- (Protein Kinases) RN - EC 2.7.1.1 (MTOR protein, human) RN - EC 2.7.10.1 (Receptor, IGF Type 1) RN - EC 2.7.11.1 (TOR Serine-Threonine Kinases) SB - IM MH - Age Factors MH - Antineoplastic Protocols MH - *Bone Neoplasms/drug therapy/radiotherapy/surgery MH - Combined Modality Therapy MH - Humans MH - Pediatrics MH - Prognosis MH - Protein Kinases/physiology MH - Receptor, IGF Type 1/physiology MH - *Sarcoma, Ewing/drug therapy/radiotherapy/surgery MH - Signal Transduction MH - TOR Serine-Threonine Kinases RF - 126 EDAT- 2008/03/19 09:00 MHDA- 2008/08/09 09:00 CRDT- 2008/03/19 09:00 PHST- 2008/03/19 09:00 [pubmed] PHST- 2008/08/09 09:00 [medline] PHST- 2008/03/19 09:00 [entrez] AID - 1024 [pii] AID - 10.2165/00148581-200810020-00004 [doi] PST - ppublish SO - Paediatr Drugs. 2008;10(2):93-105. doi: 10.2165/00148581-200810020-00004.