颅底脊索瘤的显微手术治疗探讨

探讨颅底脊索瘤的临床特点及手术治疗方案。方法回顾性分析湘雅医院神经外科2011年1月—2019年12月经显微手术治疗的33例颅底脊索瘤患者的病例资料，统计手术入路、病变切除程度及术后并发症等情况，分析患者临床疗效。结果33例患者中，全切除19例，次全切除12例，大部分切除2例，全切除率57.6%。术后有2例患者新发眼球外展受限、复视，术后1个月逐渐恢复正常；术后脑脊液鼻漏2例，均为经鼻蝶术后患者，其中1例予腰大池引流1周后拔管无脑脊液漏，1例行脑脊液漏修补术；有2例术后出现垂体功能减退，予激素替代治疗2~3个月后逐渐恢复。无围手术期死亡病例。术后随访时间6个月~108个月，19例全切除患者中有5例复发，复发率26.3%；12例次全切除患者中有4例明显进展；2例大部分切除患者中1例术后进展迅速，半年后死亡。33例患者中位无进展生存期（PFS）73个月，5年PFS 63.2%，5年生存率96.7%。结论颅底脊索瘤首选治疗方式是外科手术，术前应对其进行详细的影像学评估，选择合适的颅底手术入路尽可能提高肿瘤全切除率，术后辅助放疗可延缓肿瘤复发。

Microsurgical treatment of skull base chordoma

ObjectiveTo investigate the clinical features and surgical treatment of skull base chordoma.MethodsClinical data of 33 patients suffering from skull base chordoma microsurgically treated in our department from Jan 2011 to Dec 2019 were analyzed retrospectively. The therapeutic effect was evaluated by analysing the surgical approach, degree of tumor resection and postoperative complications.ResultsOf the 33 patients, total resection (TR) was achieved in 19, subtotal resection (STR) in 12, and most part resection in 2 with a TR rate of 57%. Two patients with new onset of limited eyeball abduction and diplopia got recovered gradually in one month after operation. Cerebrospinal fluid rhinorrhea occurred in 2 patients receiving tumor resection via trans nasal sphenoidal approach was cured with lumbar cistern drainage in one case and surgical repair in the other. Postoperative hypopituitarism in 2 patients returned to normal gradually by hormone replacement therapy for 2 to 3 months. There were no perioperative deaths. All the patients were followed up 6 to 108 months postoperatively. Tumor recurred in 5 cases after TR with a recurrence rate of 26%, and progressed significantly in 4 with STR. One of the 2 patients who underwent most part resection progressed rapidly and died half a year later. Of all the 33 cases, the median progression free survival (PFS) was 73 months with a 5 year PFS of 63.2% and a 5 year survival rate of 96.7%.ConclusionsSurgical removal is the foremost treatment method for skull base chordoma. Careful preoperative image evaluation and choice of proper surgical approach can improve the total resection rate of tumor. Postoperative adjuvant radiotherapy may postpone tumor recurrence.