Noya Carolina, D'Alessandris Quintino Giorgio, Doglietto Francesco, Pallini Roberto, Rigante Mario, Mattogno Pier Paolo, Gessi Marco, Montano Nicola, Parrilla Claudio, Galli Jacopo, Olivi Alessandro, Lauretti Liverana
School of Medicine, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.
Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo Agostino Gemelli, 8, 00168 Rome, Italy.
Cancers (Basel). 2023 Sep 9;15(18):4493. doi: 10.3390/cancers15184493.
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，给治疗带来重大挑战且预后不佳。本研究的目的是突出现有治疗方法的优缺点，提供预后指标，并阐明新的治疗策略。我们对2003年至2022年在我院接受手术治疗的斜坡脊索瘤进行了回顾性研究；为作比较，我们对已发表的手术系列进行了系统综述，最后，我们回顾了分子研究的最新进展。共有42例患者接受了85次手术；中位随访时间为15.8年，10年总生存率为49.9%；同时，10年无进展生存率为26.6%。在年轻患者（<50岁）、Ki67≤5%的肿瘤以及进行辅助放疗时，观察到生存率显著提高。总之，斜坡脊索瘤是侵袭性肿瘤，手术和放疗起着根本性作用，而分子靶向药物仍处于辅助地位。识别复发风险因素并对更具侵袭性的病变进行分子特征分析可能是未来有效治疗的关键。
