Incidence and relative survival of chordomas: the standardized mortality ratio and the impact of chordomas on a population

Abstract

Background: Chordomas are rare bone tumors arising from remnants of the embryonic notochord.

Methods: Data for this study were obtained from the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (1973-2009) to calculate the incidence, relative survival (RS), and standardized mortality ratio (SMR) of patients diagnosed with intracranial and extracranial chordomas and to assess the effects of age and sex on this disease.

Results: The overall incidence of extracranial and intracranial chordomas was 8.4 per 10 million population. The median overall survival of patients with chordoma patients was 7.7 years. The median survival was 7.7 years for male patients and 7.8 years for female patients. Younger patients (aged <40 years) survived longer compared with older patients (10-year RS, 68% vs 43%). The estimated age-standardized 5-year, 10-year, and 20-year RS rates was 72%, 48%, and 31%, respectively. The SMR in the overall cohort was 4.6 (95% confidence interval, 4.22-5.0) or 21.0 (95% confidence interval, 16.6-27.2) in young adult patients and 3.0 (95% confidence interval, 2.6-3.4) in elderly patients.

Conclusions: The elderly had a more aggressive form of this disease; and, other than the incidence, sex did not influence outcome in this disease. The study of chordomas presents a good case for the contribution that the SMR can have on measuring the impact of a disease on a population of patients. Although the younger population has better survival rates, the impact (SMR) in the younger age groups is much higher than in older populations.