Notochordal Tumors: An Update on Molecular Pathology with Therapeutic Implications.

Recent molecular investigations of chordoma show common expression of various receptor tyrosine kinases and activation of downstream signaling pathways contributing to tumor growth and progression. The transcription factor brachyury (also known as T) is important in notochord differentiation, and germline duplication of the gene is often found in familial chordomas. Nuclear expression of brachyury is consistent in chordoma and in benign notochordal cell tumor. Based on the molecular evidence, targeting of several kinds of molecular agents has been attempted for the treatment of uncontrolled chordomas and achieved partial response or stable condition in many cases.