儿童原发性中枢神经系统肿瘤763例临床分析

Primary pediatric central nervous system tumors statistic:study of 763 cases in a single institution

目的对儿童原发性中枢神经系统肿瘤的发病情况作分析调查。方法回顾性分析763例18岁以下儿童原发性中枢神经系统肿瘤病例的年龄和性别的分布情况与肿瘤的发生部位、病理学类型和分级。结果 763例患儿的平均年龄为12.7岁,男女比例为1.56:1。其中颅内肿瘤占93.4%(713/763),脊髓肿瘤占6.6%(50/763)。颅内肿瘤中,相对于幕下肿瘤(30.5%,233/763)来说,以幕上(包括松果体-四叠体区)的肿瘤(62.4%,476/763)为主。本组病例中,经病理学诊断患儿为688例,经临床诊断患儿35例。其中最常见的5种肿瘤分别为:星形细胞肿瘤(25.7%,177/688),颅咽管瘤(12.8%,88/688),髓母细胞瘤(9.3%,64/688),生殖细胞肿瘤(8.9%,61/688),垂体瘤(7.5%,52/688)。结论儿童原发性中枢神经系统肿瘤的发病具有其特殊性,对其诊断与治疗有指导性。因此,根据儿童的特点处理原发性神经系统肿瘤,能够在尽可能保留神经系统功能的情况下,综合治疗病灶。

Objective To investigate the epidemiology of primary pediatric central nervous system tumors. Methods The clinical data of 763 pediatric patients aged under 18 diagnosed as with primary central nervous system tumors in the past 10 years in Huashan Hospital were analyzed retrospectively. Results The mean age was 12.68 and the male to female ratio was 1.56： 1. Among the tumors intracranial tumors accounted for 93.4% （713/763）, whereas spinal tumors accounted for 6.6% （50/763）. 476 of the 763 tumors （62.4%） were supratentorial, including pineal-quardrigeminal tumors, which was predominant to infratentorial tumors （30.5%, 233/763）. 688 of the 763 patients had pathological diagnosis. The most common 5 categories of tumors were： astrocytic tumors （25.7% , 177/688）, craniopharyngioma （ 12.8%, 88/688）, medulloblastoma （9.3% , 64/688）, germ cell tumors （8.9%, 61/688）, and pituitary adenoma （7.5% ,52/688）. Conclusion As the main neurological medical center in the southern part of China, the statistics of Huashan Hospital can be representative. The epidemiology of pediatric central nervous system tumors has its own specialty. Therefore, it is crucial to deal with primary pediatric central nervous system tumors according to children＇s characteristics.