INTRACRANIAL CHORDOMAS - A CLINICOPATHOLOGICAL AND PROGNOSTIC STUDY OF 51 CASES

Fifty-one patients with intracranial chordomas who were surgically tre ated between 1960 and 1984 were studied. Median patient age was 46 yea rs, and 73% presented with diplopia or headache. Nineteen tumors were classified as the ''chondroid'' type. The extent of surgical removal w as a biopsy in 11 patients and subtotal removal or greater in 40. Thir ty-nine patients received postoperative radiation therapy. At the time of analysis, 17 patients were alive, and the estimated 5- and 10-year survival rates were 51% and 35%, respectively, for the group of 51 pa tients. Univariate analysis showed that: 1) patients undergoing resect ion lived longer (the 5-year survival rate was 36% for the 11 biopsy p atients compared with 55% for the 40 patients who had resection; 2) pa tients who underwent postoperative radiotherapy tended to have longer disease-free survival times; and 3) overall and disease-free survival data were the same for patients with chondroid tumors and those with t ypical chordomas. Multivariate analysis showed that: 1) age was the fa ctor most strongly associated with longer overall and disease-free sur vival: 2) diplopia was associated with longer survival; and 3) tumoral mitotic activity tended to be associated with shorter disease-free su rvival. One tumor metastasized to the cervical cord, and two tumors un derwent anaplastic transformation. These data suggest that the prognos is in patients with chordomas is unfavorable, young age is the single factor most strongly associated with longer survival, surgical resecti on is beneficial, and postoperative radiotherapy may prolong disease-f ree survival.