Skull-base Chordomas: A Systematic Review of Clinical Features, Management Approaches, and Patient Outcomes

Introduction: Chordomas are rare malignant and locally invasive bony tumors of the skull-base and spine. Despite treatment with surgical resection and post-operative radiotherapy, outcomes remain poor. We evaluated clinical factors impacting recurrence, management strategies, and patient outcomes using individual patient data in the literature.

Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a comprehensive search was conducted across PubMed, EMBASE, Web of Science, and Cochrane to identify studies including patients with histologically confirmed chordoma located in the skull-base. Clinical factors, treatment details, and patient outcomes were analyzed to identify predictor of recurrence and to characterize management strategies used.

Results: The study cohort consisted of 105 patients with skull-base chordoma. Median age was 49 years. Most patients presented with cranial nerve deficits (43%) or headaches (40%). Radiological signs of local neurovascular structure invasion were observed in 99% of patients. Both open and endonasal surgical approaches were used, with a gross total resection achieved in 49%. Recurrence occurred in 25% of patients with a median follow-up across the cohort of 17 months (range=3-96). For those that recurred, the median time to recurrence was 24 months (range=0-73). At the last follow-up, 91% of patients were alive and 9% were deceased and mean survival time was 24 months (range= 10-96 months). A significant correlation was identified between the extent of resection (gross total vs. subtotal) and recurrence (p=0.043), with a greater extent of resection correlating with fewer recurrences.

Conclusion : Skull-base chordomas are locally aggressive tumors with significant diagnostic and therapeutic challenges. This review confirms the value of current standard of care practice for skull-base chordomas while we await molecular prognostication, indicating that surgery is the mainstay of treatment and that proton beam radiotherapy has utility for reducing recurrence risk as tumor invasion into surrounding structures limits extent of resection.