Treatment of chordoma—where is it going?

Norio Yamamoto, Hiroyuki Tsuchiya


Chordomas are rare, slow-growing primary malignant tumors of the bone in which the cell of origin is derived from primitive notochordal tissues. The incidence of chordomas is estimated to be approximately 1 per 1,000,000 people (1), and they predominantly occur at the ends of the vertebral column, with approximately half of the cases occurring in the sacral region and 10% presenting in vertebral bodies. Sacral chordomas are often diagnosed late because they present with neither any significant early symptoms nor external findings. Therefore, at initial presentation, they are often detected to be quite large and at an advanced stage. In addition, chordomas are prone to recurrence and are associated with a poor prognosis.