Chordoma.

Optimal therapy of chordoma is a combined approach of surgical resection followed by proton beam irradiation for residual disease. The goals of surgery are to establish a definitive diagnosis, obtain total or maximal resection, and "shape" any residual disease so that it is favorable for radiation. Given the difficult locations and large sizes of these tumors, potential morbidity from these surgeries is high. Chordomas respond best to high doses of radiation, but nearby neurologic structures limit doses that can be delivered. Proton beam radiation appears to have a distinct advantage over conventional external beam radiation in this disease because of the rapid fall-off of radiation beyond the target. Unfortunately, the data on efficacy and safety of treatments for chordoma is limited because of the rarity of disease. Current reports in the literature are exclusively case series and reports, limiting the strength of the conclusions that can be drawn.