Chordoma

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Definition

Chordomas develop from remnants of the notochord (remnants of the notochord are usually found only in the nucleus pulposus of the intervertebral discs)

Clinical Features

80% of chordomas arise in the sacrum or the clivus (more common in the sacrococcygeal region). Sacral lesions account for 50%, and clival lesions accout for 30%.

Pathology

Consists of strands of cells with vacuolated cytoplasm (physaliphorous cells) and often contain calcium and hemorrhage.

Treatment

The tumor is radiosensitive but requires very high doses of 70-80 Gy or gamma knife with 20 Gy. The more common radiation treatment has been proton beam due to the Bragg peak allowing for high doses. Surgery is associated with high morbidity but is often required to make a diagnosis and provide symptomatic relief. In the sacrum, the treatment of choice is en bloc resection which has been shown to correlate with better disease free survival.

Left: Axial CT scan obtained in a 14-year-old boy with clival chordoma. Upper Right: Three-dimensional reconstruction of the skull base, demonstrating a massive lesion that has replaced the clivus. Lower Right: Intraoperative photograph obtained during transoral resection of clival chordoma.