H00035 | |
H number | H00035 |
Name | Ewing sarcoma |
Description | Ewing sarcoma is the second most common malignant bone tumor occurring in children and young adults, and accounts for 10-15% of all primary bone tumors. The annual incidence is approximately 0.6/million total population, and it usually occurs between the ages of 10 and 20 years. Ewing's sarcoma is in 85% of cases associated with the translocation t(11;22)(q24;q12), which leads to the formation of the EWSR1-FLI1 fusion gene. In another 10-15% of cases the translocation t(21;22)(q22;q12) generates the EWSR1-ERG fusion, whereas the remaining 1-5% of cases may harbor one of several possible translocations, each resulting in a fusion gene containing a portion of the EWSR1 gene and a member of the ETS family of transcription factors. |
Category | Cancer |
Network | - |
Gene | EWSR1-FLI1 (translocation) [HSA:2313] [KO:K09436] EWSR1-ERG (translocation) [HSA:2078] [KO:K09435] EWSR1-ETV1 (translocation) [HSA:2115] [KO:K09431] EWSR1-ETV4 (translocation) [HSA:2118] [KO:K15592] EWSR1-FEV (translocation) [HSA:54738] [KO:K09437] |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Dactinomycin [DR:D00214] |
Comment | - |
Other DBs | ICD-11: 2B52 ICD-10: C41 MeSH: D012512 |
Reference | PMID:12560386 AUTHORS Burchill SA. TITLE Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. JOURNAL J Clin Pathol 56:96-102 (2003) DOI:10.1136/jcp.56.2.96 PMID:11607824 AUTHORS Arvand A, Denny CT. TITLE Biology of EWS/ETS fusions in Ewing's family tumors. JOURNAL Oncogene 20:5747-54 (2001) DOI:10.1038/sj.onc.1204598 PMID:17250957 AUTHORS Riggi N, Stamenkovic I. TITLE The Biology of Ewing sarcoma. JOURNAL Cancer Lett 254:1-10 (2007) DOI:10.1016/j.canlet.2006.12.009 PMID:16096383 AUTHORS Khoury JD. TITLE Ewing sarcoma family of tumors. JOURNAL Adv Anat Pathol 12:212-20 (2005) DOI:10.1097/01.pap.0000175114.55541.52 PMID:12633526 AUTHORS Riley RD, Burchill SA, Abrams KR, Heney D, Lambert PC, Jones DR, Sutton AJ, Young B, Wailoo AJ, Lewis IJ. TITLE A systematic review and evaluation of the use of tumour markers in paediatric oncology: Ewing's sarcoma and neuroblastoma. JOURNAL Health Technol Assess 7:1-162 (2003) DOI:10.3310/hta7050 |