Abstract
BACKGROUND: Chordoma is the most common primary malignant tumor of the spine. It is extremely rare and has been studied primarily in single-institution case series. Using data from a large, population-based cancer registry, we designed the present study to examine the outcome for patients with chordoma and to determine relevant prognostic factors. METHODS: A retrospective analysis of the California Cancer Registry database was performed to identify patients with a diagnosis of chordoma in the years 1989 to 2007. Comparisons examined differences in demographics, disease characteristics, treatment, and survival. Survival analyses were performed with use of the Kaplan-Meier method with log-rank tests and Cox proportional hazards models. RESULTS: Four hundred and nine patients with chordoma were identified; 257 (62.8%) were male and 152 (37.2%) were female. With regard to racial or ethnic distribution, 266 patients (65%) were white; ninety-three (22.7%), Hispanic; forty-three (10.5%), Asian or other; and seven (1.7%), black. The site of presentation was the head in 202 patients (49.4%), spine in 106 patients (25.9%), and pelvis and/or sacrum in 101 patients (24.7%). Hispanic race (p = 0.0002), younger age (less than forty years; p < 0.0001), and female sex (p = 0.009) were associated with cranial presentation, whereas older age (forty years or older; p < 0.0001) was associated with pelvic presentation. After adjustment for clinically relevant factors, a significantly decreased risk of death for chordoma-specific survival was seen for Hispanic race (hazard ratio = 0.51, 95% confidence interval [95% CI], 0.28 to 0.93; p = 0.03), high socioeconomic status (hazard ratio = 0.8, 95% CI, 0.67 to 0.95; p = 0.01), and local excision and/or debulking (hazard ratio = 0.38, 95% CI, 0.18 to 0.81; p = 0.01). Large tumor size was independently associated with an increased risk of death (hazard ratio = 2.05, 95% CI, 1.01 to 4.20; p = 0.048). CONCLUSIONS: In this study, the survival of patients with chordoma was significantly better for those who were Hispanic and had a small tumor, high socioeconomic status, and surgical intervention.