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.
斜坡脊索瘤是罕见但具有侵袭性的颅底肿瘤，治疗难度大且预后不良。本研究旨在阐明现有治疗方法的优势与局限，提供预后指标，并探讨新型治疗策略。我们对2003年至2022年间在本机构接受手术治疗的斜坡脊索瘤病例进行回顾性研究；为便于比较，我们对已发表的手术系列研究进行了系统性综述，并梳理了分子研究领域的最新进展。42例患者共接受85次手术，中位随访时间15.8年，10年总生存率为49.9%，10年无进展生存率为26.6%。年龄较轻（<50岁）、Ki67指数≤5%以及接受辅助放疗的患者生存率显著改善。综上所述，斜坡脊索瘤作为侵袭性肿瘤，手术与放疗仍发挥核心作用，而分子靶向药物目前仅处于辅助地位。识别肿瘤复发的危险因素并对高侵袭性病变进行分子特征分析，可能是未来实现有效治疗的关键。
Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review
肿瘤(癌症)患者之家