These are rare malignant tumors which are found at the spine and sometimes at the base of the skull. The tumor is of malignant variety and is considered as a rare because less than 1% of central nervous system tumors.
Much as they are slow-growing, chordomas are locally aggressive and tend to infiltrate adjacent organs and tissues. This recurrence locally leads to destruction of tissue and can cause death.
As primary intracranial tumors, chordomas constitute of 0.2% of all the CNS tumors and constitute 2-4% of all primary bone neoplasms.
Chordomas are known to generally occur in 3 places:
Intracranially at the clivus
Male to female ratio is 2:1. Skull base tumors however tend to end up with more equal distribution in terms of sex. Reports show that chordomas affect all age groups and incidence only varies with the site. Intracranial chordomas are more common in younger age groups.
Average age of Occurrences:
Sphenoccipital is 38 years.
Sacrococcygeal chordomas-56 years.
Along the vertebrae is 46 years.
In the United States, annual chordoma incidence is 1 in every million.