Research Advances in Molecular Regulatory Mechanism of Chordoma

Chordoma is a rare primary malignant bone tumor derived from notochord remnants. Due to its strong local invasiveness and chemotherapy resistance, chordoma has a high local recurrence rate and poor prognosis. Although previous studies identified many molecules including brachyury as the oncogenes in chordoma, the pathogenesis of chordoma remains to be fully elucidated. The treatment of chordoma is still a challenge for the surgeons. In recent year, multiple novel biomarkers such as iASPP, SMARCB1 and signaling pathways like PI3K/AKT have been reported to be related with the pathogenesis of chordoma. Identifying crucial targets and signaling pathways is helpful to understand the pathogenesis of chordoma further, and lays a solid foundation to develop new drugs to improve the therapeutic effects of chordoma on the other hand. In this paper, we aim to review the recent advances in molecular regulation mechanism of chordoma.