Outcomes and Patterns of Care in Adult Skull Base Chordoma and Chondrosarcoma from the Surveillance, Epidemiology, and End Results (SEER) Database

Abstract

Objective: This study aims to demonstrate survival rates and treatment patterns among patients with chordomas of the skull base using a large-population database. Methods: Patients with cranial chordomas between 1973 and 2009 were identified from the SEER Public-Use database. Kaplan-Meier analysis was used to examine the effect of surgery and radiation on overall survival. Results: Chordoma: 394 patients had histologically-confirmed cranial chordomas. Median survival was 151 months. Most patients (89.09%) underwent surgery. Less than half (44.92%) received radiation after diagnosis. Patients who underwent surgical resection survived significantly longer than those who did not undergo resection, regardless of other treatments (151 versus 81 months, p < 0.001). Ten-year survival was lower among patients receiving radiation (44.8% versus 61.4%, p = 0.66). Surgery predicted better overall survival by univariate analysis (Hazard ratio (HR) 0.603, p = 0.0293); younger age at diagnosis (HR 1.028, p < 0.001), and later year of diagnosis (HR 0.971, p = 0.0027) were prognostic of improved survival in a multivariate model. In subgroup analysis of cases with documented tumor size, smaller tumor size (HR 1.021, p = 0.0067), younger age (HR 1.031, p = 0.001), and treatment within a higher volume registry (HR 0.490, p = 0.0129) predicted improved survival. Chondrosarcoma: 226 patients had skull base chondrosarcomas. Median overall survival was 264 months, 10-year survival 68.2%. Most patients underwent surgery (92.5%). Few received radiation after diagnosis (38.1%), with no significant increasing trend over time (p = 0.9). Ten-year survival for all patients treated with surgery was significantly increased compared with those without surgery, other than biopsy (69.3% versus 53.9%, p = 0.02). Median overall survival for patients who received surgery and radiation was not reached at time of last data collection, compared with 264 months for non-irradiated surgical patients (p = 0.07). Surgery predicted better overall survival by univariate analysis (Hazard ratio (HR) 0.420, p = 0.0247); female gender (HR 0.470, p = 0.011), younger age at diagnosis (HR 1.046, p < 0.0001), and later year of diagnosis (HR 0.949, p = 0.0006) were prognostic of improved survival in a multivariate model. In subgroup analysis of cases with documented tumor size, smaller tumor size (HR 1.054, p = 0.0003) and younger age (HR 1.021, p = 0.0067) predicted improved survival. Conclusions: Surgical intervention offers survival benefit for cranial chordomas. For chordrosarcoma, the combination of surgery and radiotherapy appears to confer the best outcome. Although radiation prolonged chondrosarcoma median survival, it was used in a minority of cases, underscoring the need for better definition of effective treatment of this disease.