Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas
Article first published online: 14 MAR 2006
Copyright © 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
The Journal of Pathology
Volume 209, Issue 2, pages 157–165, June 2006
How to Cite
Vujovic, S., Henderson, S., Presneau, N., Odell, E., Jacques, T., Tirabosco, R., Boshoff, C. and Flanagan, A. (2006), Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J. Pathol., 209: 157–165. doi: 10.1002/path.1969
- Issue published online: 11 MAY 2006
- Article first published online: 14 MAR 2006
- Manuscript Accepted: 11 JAN 2006
- Manuscript Revised: 19 DEC 2005
- Manuscript Received: 22 NOV 2005
- Cancer Research UK
- Wellcome Trust, UK
- nucleus pulposus
Chordomas are malignant tumours that occur along the spine and are thought to derive from notochordal remnants. There is significant morphological variability between and within chordomas, with some showing prominent areas of chondroid differentiation. Our microarray data from a broad range of connective tissue neoplasms indicate that, at the transcriptional level, chordomas resemble cartilaginous neoplasms. Here we show that chordomas express many genes known to be involved in cartilage development, but they also uniquely express genes distinguishing them from chondroid neoplasms. The brachyury transcription factor, known to be involved in notochordal development, is only expressed by chordomas. Using a polyclonal antibody, we show that brachyury is expressed in the embryonic notochord and in all 53 chordomas analysed, labelling both chondroid and chordoid areas of these tumours. In contrast, the protein was not detected in over 300 neoplasms, including 163 chondroid tumours. Brachyury was not detected in the nucleus pulposus, arguing against the hypothesis that this tissue derives directly from the notochord. These data provide compelling evidence that chordomas derive from notochord and demonstrate that brachyury is a specific marker for the notochord and notochord-derived tumours. Copyright © 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.