Ewing sarcoma: a chronicle of molecular pathogenesis

Hum Pathol. 2016 Sep:55:91-100. doi: 10.1016/j.humpath.2016.05.008. Epub 2016 May 28.

Abstract

Sarcomas have traditionally been classified according to their chromosomal alterations regardless of whether they accompany simple or complex genetic changes. Ewing sarcoma, a classic small round cell bone tumor, is a well-known mesenchymal malignancy that results from simple sarcoma-specific genetic alterations. The genetic alterations are translocations between genes of the TET/FET family (TLS/FUS, EWSR1, and TAF15) and genes of the E26 transformation-specific (ETS) family. In this review, we intend to summarize a chronicle of molecular findings of Ewing sarcoma including recent advances and explain resultant molecular pathogenesis.

Keywords: Chromosomal translocation; Ewing sarcoma; Molecular pathogenesis.

Publication types

  • Review

MeSH terms

  • 12E7 Antigen / analysis
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics*
  • Bone Neoplasms / chemistry
  • Bone Neoplasms / genetics*
  • Bone Neoplasms / pathology
  • Chromosome Aberrations
  • Diagnosis, Differential
  • Epigenesis, Genetic
  • Gene Expression Regulation, Neoplastic
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Molecular Diagnostic Techniques
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Sarcoma, Ewing / chemistry
  • Sarcoma, Ewing / genetics*
  • Sarcoma, Ewing / pathology
  • Transcription, Genetic
  • Translocation, Genetic

Substances

  • 12E7 Antigen
  • Biomarkers, Tumor
  • CD99 protein, human