Chordoma

Excerpt

A chordoma is a low-grade, slow-growing, but locally invasive and locally aggressive tumor. Chordomas belong to the sarcoma family of tumors. They arise from the remnants of the notochord and occur in the midline along the spinal axis from the clivus to the sacrum, anterior to the spinal cord. The location distribution of chordomas is 50% sacral, 35% skull base, and 15% occur in the vertebral bodies of the mobile spine (most commonly the C2 vertebrae followed by the lumbar then thoracic spine). Overall 5-year survival is approximately 50%, and treatment is en bloc surgical resection followed by high-dose conformal radiation therapy such as proton beam radiation.