Management Strategies in Clival and Craniovertebral Junction Chordomas: A 29-Year Experience

Objective: Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive surgical resection followed by proton beam therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. The aim of this study is to present our experience in treating clival and CVJ chordomas over a 29-year period.

Methods: We conducted a retrospective study of clival and CVJ chordomas operated in our institution from 1991 to 2020. This study focused on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEA) compared with open approaches (OA).

Results: A total of 265 surgical procedures were performed on 210 patients, including 123 (46.4%) OA and 142 (53.6%) EEA. Patients whose tumor had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003) or a high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross total resection (GTR) and K_i-67 LI <6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative RT correlated independently with a longer PFS (p = 0.006). The previously operated patients’ group was associated with a lower EOR (p = 0.01), a higher rate of CSF leakage after EEA (p = 0.02), but did not have significantly lower PFS and OS than the primary patients group. Previously irradiated patients correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEA was more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003) and a lower risk of de novo neurological deficit (p < 0.0001) compared with OA. The overall rate of postoperative CSF leakage after EEA was 14.8%.

Conclusions: This large study showed that GTR should be attempted in a multidisciplinary skull base center before providing radiotherapy. EEA should be considered as the gold standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OA remain important tools for large complex chordomas.

Publication History

Article published online:
01 February 2023