Brachyury and chordoma: the chondroid-chordoid dilemma resolved?

J Pathol. 2006 Jun;209(2):143-6. doi: 10.1002/path.1987.


Chordoma, and its relationship to the notochord, has intrigued many researchers over the last two centuries. In particular, the morphological overlap with cartilaginous tumours is striking, and developmental biology has shown a tight relationship between cartilage and the notochord. This is reflected in the expression of common genes in chordoid and chondroid tumours. Wide gene expression analyses have led to the identification of key molecules that might play a crucial role in the pathogenesis of chordoma. Brachyury, a key factor in notochord fate, is significantly differentially expressed in chordoma. This not only gives insight into the histogenesis of this tumour but may also point towards new diagnostic tools in the differential diagnosis between chordoid and chondroid tumours.

MeSH terms

  • Bone Neoplasms / embryology
  • Bone Neoplasms / genetics
  • Bone Neoplasms / pathology
  • Cartilage Diseases / embryology
  • Cartilage Diseases / genetics*
  • Chordoma / embryology
  • Chordoma / genetics*
  • Chordoma / pathology
  • Diagnosis, Differential
  • Fetal Diseases / genetics
  • Fetal Diseases / pathology
  • Fetal Proteins / genetics*
  • Gene Expression Regulation, Neoplastic / genetics
  • Humans
  • Neoplasm Proteins / genetics
  • Notochord / embryology
  • Notochord / pathology*
  • T-Box Domain Proteins / genetics*


  • Fetal Proteins
  • Neoplasm Proteins
  • T-Box Domain Proteins
  • Brachyury protein