KEGG   DISEASE: Ewing sarcomaHelp
Entry
H00035                      Disease                                

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;12)(22;12) 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
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H00035  Ewing sarcoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant mesenchymal neoplasms
     2B52  Ewing sarcoma, primary site
      H00035  Ewing sarcoma
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00133  EWSR1-FLI1 fusion to transcriptional activation
N00134  EWSR1-FLI1 fusion to transcriptional repression
N00135  EWSR1-ERG fusion to transcriptional activation
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]
Drug
Dactinomycin [DR:D00214]
Comment
ICD-O: 9260/3, Tumor type: Ewing sarcoma
Other DBs
ICD-11: 2B52
ICD-10: C41
MeSH: D012512
Reference
PMID:12560386 (description, gene, tumor type)
  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
Reference
  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
Reference
  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
Reference
PMID:16096383 (marker)
  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
Reference
PMID:12633526 (marker)
  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

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