Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review

SIMPLE SUMMARY: We report the experience of our institution in treating clival chordomas over 20 years and systematically review the recent literature, highlighting factors associated with outcome (age < 50 years, Ki67 ≤ 5%, and adjuvant radiotherapy are associated with better overall survival) and clues for new therapies. Recurrence in clival chordomas remains part of the disease history despite maximal treatment. Still, significant variations are evident in overall and progression-free survival, highlighting the need to develop efficient treatment strategies and recognize which factors reliably predict a more aggressive behavior of clival chordomas. ABSTRACT: Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research. A total of 42 patients underwent 85 surgeries; median follow-up was 15.8 years, overall survival rate was 49.9% at 10 years; meanwhile, progression-free survival was 26.6% at 10 years. A significantly improved survival was observed in younger patients (<50 years), in tumors with Ki67 ≤ 5% and when adjuvant radiotherapy was performed. To conclude, clival chordomas are aggressive tumors in which surgery and radiotherapy play a fundamental role while molecular targeted drugs still have an ancillary position. Recognizing risk factors for recurrence and performing a molecular characterization of more aggressive lesions may be the key to future effective treatment.