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.
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.
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).
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.
Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.
1Department of Orthopaedic Surgery (J.L., N.N.B., and B.H.H.), Spine Center (N.N.B.), Chao Family Comprehensive Cancer Center (B.H.H.), University of California, Irvine, 101 The City Drive South, Pavilion 3, Orange, CA 92868
2Department of Epidemiology, School of Medicine (A.Z. and J.A.Z.), and the Genetic Epidemiology Research Institute (A.Z.), University of California, Irvine, 224 Irvine Hall, Irvine, CA 92697-7550. E-mail address for J.A. Zell: email@example.com