title: Analysis of prognostic factors for patients with chordoma with use of the California Cancer Registry.
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.
authors.affiliation: Department of Orthopaedic Surgery, University of California, Irvine, Irvine, California, USA.
authors.forename: Joe
authors.initials: J
authors.lastname: Lee
authors.forename: Nitin N
authors.initials: NN
authors.lastname: Bhatia
authors.forename: Bang H
authors.initials: BH
authors.lastname: Hoang
authors.forename: Argyrios
authors.initials: A
authors.lastname: Ziogas
authors.forename: Jason A
authors.initials: JA
authors.lastname: Zell
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002140:California; D002648:Child; D002675:Child, Preschool; D002817:Chordoma / Q000175:diagnosis / Q000401:mortality*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000175:diagnosis / Q000401:mortality*; D015996:Survival Rate; D055815:Young Adult