483例儿童中枢神经系统肿瘤临床及病理特点分析

Clinicopathological characteristics of central nervous system tumors in 483 children

目的:总结儿童中枢神经系统(CNS)肿瘤临床及病理特点,提高大家对儿童CNS肿瘤诊治的认识。方法:收集2015年1月至2021年6月在广州市妇女儿童医疗中心神经外科接受手术治疗的483例儿童CNS肿瘤患者临床资料,详细分析其性别、年龄、临床表现、肿瘤部位及病理类型等。结果:483例患者中,男289例(59.83%),女194例(40.17%),男女比例为1.49∶1,年龄在1月~14岁之间,其中≤3岁264例(54.66%),≤7岁399例(82.60%),>7岁的仅为84例(17.40%)。临床表现多样,其中呕吐105例(21.74%),头痛40例(8.28%),肢体乏力59例(12.21%),行走不稳80例(16.56%)。483例患者均接受手术治疗,其中颅内肿瘤410例(84.89%),椎管内肿瘤73例(15.11%)。颅内肿瘤中,幕上249例(60.73%),幕下161例(39.27%);中线部位257例(62.68%),非中线部位153例(37.32%)。术后病理类型排名前5位依次是:星形细胞肿瘤132例(27.33%),髓母细胞瘤56例(11.59%),生殖细胞肿瘤53例(10.97%),非典型畸胎瘤样/横纹肌样瘤31例(6.42%),颅咽管瘤29例(6.00%),术前影像学诊断符合率仅为40%。结论:儿童CNS肿瘤与成人明显不同,早期临床症状不典型,误诊率高。病理类型多样,高级别肿瘤比例随年龄增长而增加。正确认识儿童CNS肿瘤临床病理特点,有利于其下一步个体化治疗方案的制定。

Objective:To summarize the clinicopathological characteristics of central nervous system(CNS)tumors in children,and to improve understanding on diagnosis and treatment of CNS tumors in children.Methods:The clinical data of 483 children with CNS tumors undergoing surgery in the Department of Neurosurgery,Guangzhou Women and Children’s Medical Center between January 2015 and June 2021,were included in the study.The patient gender,age,clinical manifestations,tumor site and pathological type were analyzed in details.Results:Among the 483 patients,there were 289 males(59.83%)and 194 females(40.17%),with a male⁃to⁃female ratio of 1.49∶1.The age ranged from 1 month to 14 years old,including 264 children(54.66%)aged≤3 years,399(82.60%)≤7 years,and only 84(17.40%)>7 years.The clinical manifestations were diverse,including vomiting in 105(21.74%)cases,headache in 40(8.28%),limb weakness in 59(12.21%),and dystaxia in 80(16.56%).All 483 patients underwent surgery for intracranial tumors[n=410(84.89%)]or intraspinal tumors[n=73(15.11%)].The sites of intracranial tumors were supratentorial in 249(60.73%),infratentorial in 161(39.27%),midline in 257(62.68%),and non⁃midline in 153(37.32%)children.The first 5 leading pathological types identified after surgery were:132 cases(27.33%)of astrocytoma,56(11.59%)of medulloblastoma,53(10.97%)of germ cell tumor,31(6.42%)of atypical teratoid/rhabdoid tumor,and 29(6%)of craniopharyngioma.The coincidence rate of preoperative imaging diagnosis was only 40%.Conclusion:CNS tumors in children differ significantly from those in adults,with atypical clinical symptoms in the early stage and a high misdiagnosis rate.The pathological types are diverse,with higher proportion of high⁃grade tumors along with elder age.Understanding the clinical and pathological characteristics of CNS tumors in children may favor subsequent development of individualized treatment protocol.