Chordoma: an update on the pathophysiology and molecular mechanisms.
Chordoma is a rare low-grade primary malignant skeletal tumor, which is presumed to derive from notochord remnants. The pathogenesis of chordoma has not been fully elucidated. However, recent advances in the molecular biology studies have identified brachyury underlying the initiation and progression of chordoma cells. More efforts have been made on accumulating evidence of the notochordal origin of chordoma, discovering signaling pathways and identifying crucial targets in chordomagenesis. In this review, we summarize the most recent research findings and focus on the pathophysiology and molecular mechanisms of chordoma.
Section of Orthopedic Oncology, Department of Orthopedic Surgery, Harvard Medical School, Massachusetts General Hospital, Yawkey 355 Fruit Street, Boston, MA, 02114, USA.
Xin
X
Sun
Section of Orthopedic Oncology, Department of Orthopedic Surgery, Harvard Medical School, Massachusetts General Hospital, Yawkey 355 Fruit Street, Boston, MA, 02114, USA.
Francis
F
Hornicek
Section of Orthopedic Oncology, Department of Orthopedic Surgery, Harvard Medical School, Massachusetts General Hospital, Yawkey 355 Fruit Street, Boston, MA, 02114, USA. JHSCHWAB@mgh.harvard.edu.
Joseph H
JH
Schwab
Chordoma; Chromosomal alterations; DNA methylation; Pathophysiology; microRNA