【摘要】 目的:阐述儿童及青少年颅底脊索瘤的临床特征及治疗方法,并分析影响其预后的因素。方法:对12例儿童及青少年颅底脊索瘤的临床表现、影像学特征、病理类型、手术及放射治疗等临床资料进行回顾性分析。结果:12例患者中,男性3例、女性9例,男女性别比为1:3;发病年龄5~17岁,平均13.2岁。呕吐、头痛及复视为最常见的临床表现。病变多发于斜坡-后颅窝,多有斜坡骨质破坏。病理检查典型脊索瘤10例,软骨样脊索瘤2例。12例患者共行14次手术,首次手术5例全切,5例次全切,2例部分切除。随访6 d至78个月(平均31.5个月),不同年龄、手术切除程度和是否接受放疗对患者的生存期有影响,差异有统计学意义(P<0.05)。结论:在儿童及青少年颅底脊索瘤患者中,年龄较大者(10~18岁)具有较好的预后生存期,手术全切及放疗为相对有效的治疗方法。更多还原

【Abstract】 Aim:To elucidate clinical features and management of skull base chordomas in children and adolescents,and to evaluate the factors affecting the long-term survivals.Methods: Clinical data of 12 children and adolescents with skull base chordomas,who were surgically treated in a single institution over a 20-year period,were retrospectively reviewed and analyzed.Results: There were 3 males and 9 females,aged from 5 to 17 years old,mean 13.2 years old.Vomiting,headache and diplopia were the most frequent symptoms or signs.Clivus and posterior cranial fossa were most involved,and bone destruction was noted usually.Conventional and chondroid chordomas comprised 83.3% and 16.7% of all cases,respectively.A total of 14 procedures were performed in patients.In the first procedure,5 patients received total resection,5 patients subtotal resection and 2 patients partial resection.Follow-up data were available for all the patients.The period ranged from 6 days to 78 months(mean 31.5 months).There were statistical significant differences in survival between younger group(<10 years) and older group(>10 years)(P=0.009),radiotherapy group and non-radiotherapy group(P=0.0468).And so was observed among total resection group,subtotal resection group and partial resection group(P=0.039,P=0.002,P=0.048).Conclusion: For children and adolescents with skull base chordomas,older patients have a significantly better outcome in survival than younger ones.Radical resection of tumors and radiotherapy are usually regarded as the most efficacious treatments.更多还原