Chordoma is a rare type of tumor of the skeletal system that can occur anywhere along the spine, from the base
of the skull to the tailbone. Chordoma is a slowly growing primary tumor that arise from an intra-osseous remnant of
notochordal cells. These neoplasms typically occur in the axial skeleton; the most common location is the sacrococcygeal
region followed by skull base and spine. Chordomas typically occur in adults between ages 50 and 70. About five percent
of them are diagnosed in children. Males are affected about twice as often as females.
Histopathologically, chordomas are divided into conventional (the most common) type, chondroid, and dedifferentiated
types. They are mainly locally aggressive with gradual involvement of bone and soft tissues. However, metastases have
been reported mainly at late stages. Aggressive initial therapy, based on maximal possible resection followed by postoperative
irradiation, improves overall outcome.
Up to 40 percent of patients recur after treatment showing a poor prognosis but both radiation and surgery can be used as
salvage therapy. Radiation therapy is used in order to treat patients with advanced, residual, inoperable lesions or with
local recurrence. Particle therapy (protons and ions) has been reported as a very active form of irradiation for its peculiar
physical properties. Chordomas are reported as tumors non sensitive to chemotherapy; molecularly targeted therapies are
increasingly used in recent years with promising results but deserve further investigation.