66例颅底脊索瘤的临床诊治和预后分析

Clinical diagnosis and prognosis of skull base chordomas in 66 patients

目的探讨颅底脊索瘤的临床特点、诊断、治疗和预后。方法回顾性分析2000年1月～2010年1月接受手术治疗且有完整随访资料的66例颅底脊索瘤,并分析其临床症状、体征、影像学检查及手术径路。结果 66例颅底脊索瘤患者头痛、视力受损症状最为常见。肿瘤全切或基本全切除25例(32.5%),次全切除32例(47.6%),部分切除9例(11.7%)。术后28例患者辅助性接受伽马刀治疗。3,5年无进展生存率分别为82.6%和45%;3,5年的总体生存率分别为89.2%和70.9%。结论目前脊索瘤手术全切除率不高,容易复发,预后差,较彻底的手术切除结合伽马刀治疗能使颅底脊索瘤取得相对满意的疗效。

Objective To analyze the clinical characteristics, diagnosis, treatment and outcome of skull base chordomas. Methods Clinical data of 66 patients suffering from skull base chordomas and treated surgically with full follow-up was analyzed retrospectively. Results The mean age of 66 patients was 39, ranging from 5 to 67. 40 patients were male and the other 26 were female. Headache, visual disturbance and abducent nerve palsy were the major symptoms or signs. Total tumor resection was achieved in 25 cases （32.5%） , subtotal in 32 （47.6%） and partial in 9 （ 11.7% ）. Gamma knife radiosurgery （GKR） was applied to 28 patients （33. 3% ）. The progress-free survival rates at 3 and 5 years were 82.6% and 45.0% respectively. The overall survival rates at 3 and 5 years were 89.2 % and 70.9 % .Conclusion Aggressive surgial treatment is suitable for skull base chordoma with low total resection, high recurrence and poor outcome. Favourable outcome for skull base chordomas can be achieved with the combination of aggressive surgical resection and GKR.