Current Management of Chordoma

Chordomas are relatively rare, slowly growing primary tumors of bone that arise from embryonic remnants of the notochord. These neoplasms typically occur in the axial skeleton mainly in the skull base (about 35%) and sacral region (about 50%) and have an overall peak distribution in patients aged 55-65 years. Chordomas are divided into conventional (the most common type), chondroid, and dedifferentiated types. Aggressive initial therapy, based on radical-maximal surgery followed by post-operative irradiation, improves overall outcome. Patients who relapse locally have a poor prognosis but both radiation and surgery can be used as salvage therapy. Radiation therapy alone may also be used in order to treat some patients with advanced, inoperable lesions or local recurrence. Proton beam irradiation has been reported as a very active form of irradiation for its peculiar physical properties. Chordomas are reported as tumors non sensitive to chemotherapy, even though anecdotal reports have been shown some sensitivity to molecularly targeted therapies.