DISEASE: Ewing sarcoma
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.
Human diseases [BR:
Cancers of soft tissues and bone
H00035 Ewing sarcoma
Human diseases in ICD-11 classification [BR:
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
Transcriptional misregulation in cancer
EWSR1-FLI1 fusion to transcriptional activation
EWSR1-FLI1 fusion to transcriptional repression
EWSR1-ERG fusion to transcriptional activation
EWSR1-FLI1 (translocation) [HSA:
EWSR1-ERG (translocation) [HSA:
EWSR1-ETV1 (translocation) [HSA:
EWSR1-ETV4 (translocation) [HSA:
EWSR1-FEV (translocation) [HSA:
ICD-O: 9260/3, Tumor type: Ewing sarcoma
(description, gene, tumor type)
Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities.
J Clin Pathol 56:96-102 (2003)
Arvand A, Denny CT.
Biology of EWS/ETS fusions in Ewing's family tumors.
Oncogene 20:5747-54 (2001)
Riggi N, Stamenkovic I.
The Biology of Ewing sarcoma.
Cancer Lett 254:1-10 (2007)
Ewing sarcoma family of tumors.
Adv Anat Pathol 12:212-20 (2005)
Riley RD, Burchill SA, Abrams KR, Heney D, Lambert PC, Jones DR, Sutton AJ, Young B, Wailoo AJ, Lewis IJ.
A systematic review and evaluation of the use of tumour markers in paediatric oncology: Ewing's sarcoma and neuroblastoma.
Health Technol Assess 7:1-162 (2003)
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