Surgery significantly improves survival in patients with chordoma.

Study Design: Retrospective database review using Surveillance, Epidemiology, and End Results database, National Cancer Institute.

Objective: To determine current treatment outcomes and demographic characteristics and to define the prognostic factors and impact of surgery on survival.

Summary Of The Background Data: Rarity of the disease has limited the number of population-based studies addressing the issues of prognostic factors and the current treatment outcomes.

Methods: A total of 962 patients with chordoma, diagnosed between 1973 and 2005, were identified in the Surveillance, Epidemiology, and End Results database. Patient demographics and clinical characteristics, incidence, year of diagnosis, location, size and stage at diagnosis, treatment(s), and survival were extracted. Kaplan-Meier, log-rank, and Cox regression were used to analyze the significance of prognostic factors.

Results: Race specific incidence per 100,000 persons in 2005 shows whites to have the highest (0.473) incidence, followed by Asians/Pacific Islanders (0.091), and Afro-Americans (zero), respectively. The incidence difference between whites and Afro-Americans over time is statistically significant (P < 0.001). The age adjusted incidence reveals a peak in eighth decade of life. Survival was not impacted by race. Age category <59, Hispanic ethnicity, size <8 cm, and surgical resection were all independent predictors of better overall survival. Analysis revealed that survival outcomes were very similar in patients with "in-operable disease" and those in which "surgery was recommended but not performed" suggesting that benefits of resection cannot be solely attributed to selection bias.

Conclusion: With high local recurrence rates and the significant functional morbidity associated with surgical resections, clinicians are left wondering if surgery constitutes an appropriate treatment option. Our study clearly demonstrates that surgery significantly improves the overall survival for patients with chordoma. We have further identified age <59 and size of primary tumor <8 cm as important prognostic factors determining the outcome with substantial statistical significance.