Disease outcomes for skull base and spinal chordomas: a single center experience.

OBJECTIVE: Chordomas carry significant morbidity due to their growth patterns and surgical constraints in resection. En bloc resection, when feasible, is the ideal treatment goal, but is associated with significant morbidity. We sought to elucidate the relationship between extent of surgery, location and radiotherapy in relation to overall disease and progression free survival (PFS).

METHODS: We reviewed case records for all patients with a primary histopathological diagnosis of clival and spinal chordomas that was presented to our institution between 1978 and 2010.

RESULTS: A total of 49 patients (location: n=30, skull base/clival; n=12 vertebral column; n=7 sacrum) were identified with mean follow-up period of 6.3 years (range 0.25 months-33 years). Improved 5 year and 10 year survival rates were noted following gross total resection (n=8, 5 year and 10 year survival=88%) as compared to patients that underwent subtotal resection (n=41, 55% and 31%, respectively), (p-value>0.05, GTR versus STR). Adjuvant high-dose stereotactic fractionated radiotherapy (HS-FSRT) significantly improved 5 year PFS in craniocervical chordoma patients (70%, n=13) as compared to standard dose radiation therapy (20%, n=16; p-value=0.03). Overall 10 year survival for craniocervical patients undergoing HD-FSRT (40%) was however not significantly different in comparison with conventional radiotherapy (45%). Sacral chordomas had the worst prognosis with 3 year survival of 28.6%.

CONCLUSIONS: GTR offers the best prognosis for improved long-term survival. Adjuvant HD FSRT for cranio-cervical/clival chordomas significantly improves disease free survival though the long-term benefits on survival have yet to be established. Sacral chordomas are associated with a worse prognosis and poor long-term survival.