Chordoma

Epidemiology

Chordomas are rare tumors that account for 3 to 4% of primary malignant bone tumors and are the most common primary sacral neoplasm. Chordomas can occur at any age, but the peak incidence is in the sixth decade. There is a 2:1 male predominance. These tumors can occur anywhere along the skull base and spine: 50% arise in the sacrum, 35% in the clivus, and 15% in the vertebrae. In the spine, the areas most commonly involved are the cervical, lumbar, and thoracic spine, in descending order of frequency. Chordomas are locally invasive tumors with no propensity for metastatic spread. However, chordomas arising in the vertebral bodies have a greater likelihood for distant metastases than their counterparts in the sacrum or the clivus. Although metastases have been reported in 10 to 15% of all cases, metastases occur in 80% of chordomas arising in the vertebral bodies.