Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients.

The clinical presentation and the results of operative and radiation therapy in a series of 26 patients with cranial chordomas seen at the University of California, San Francisco, between 1940 and 1984 are reviewed. There were 14 men and 12 women, with a mean age of 39.6 years. Six patients had chondroid chordomas. The most common presenting symptoms were headache and diplopia, and the most common presenting sign was extraocular palsy. Fifty-three operations directed at removal of the tumor were performed. Twenty-three patients received various forms of radiation therapy postoperatively, including conventional external beam therapy, heavy charged particles, and interstitial implants. The average length of follow-up is 5.6 years. Eleven of 26 patients have died; the mean duration of survival in this group, excluding 1 perioperative death, was 4 years and 2 months. Although the average survival time of 6 years and 4 months was the same in patients with typical chordomas (excluding the perioperative death and 1 patient lost to follow-up) and in those with the chondroid variant, all of the latter are still alive, whereas more than half of the patients with typical chordoma have died.