儿童中枢神经系统肿瘤398例临床病理分析

Pediatric tumors of nervous system tumors: a clinicopathologic study of 398 cases

目的分析儿童中枢神经系统肿瘤(CNS)的临床特点和病理类型及之间的关系,以提高病理诊断的准确性。方法按照WHO(2016)中枢神经系统肿瘤分类标准,对398例16岁儿童中枢神经系统肿瘤的组织病理学特点、肿瘤部位、患者年龄、性别、族别等临床特征进行汇总分析。结果 398例中,肿瘤位于幕上228例(57.3%),幕下147例(37%),椎管内23例(5.8%)。患儿平均年龄8.99岁,其中≤3岁54例(13.6%),4~9岁157例(39.4%),10~16岁187例(47%);随着年龄的增长发病比例逐渐增高,男女比例为1.1∶1。病理类型前5位分别是星形细胞肿瘤88例(22.1%),髓母细胞瘤86例(21.6%),颅咽管瘤51例(12.8%),室管膜肿瘤42例(10.6%),生殖细胞肿瘤19例(4.8%)。结论新疆地区儿童中枢神经系统肿瘤中,幕上发病率高于幕下,男女比例相当,肿瘤的组织病理学类型与患者的年龄、性别、部位等因素有关,全面了解其特点对正确的病理诊断及临床治疗均有重要意义。

Objective To analyze the clinical features and pathological types of central nervous system tumor of children in Xinjiang,to improve the accuracy of pathological diagnosis,and to analyze the relationship between pathology and clinic.Methods The clinical and pathological data were collected and analyzed in 398 cases of children under 16 years old on the histopathological characteristics of the central nervous system tumors in the First Affiliated Hospital of Xinjiang Medical University from 2008 to 2016,including tumor location,patient age,gender and other clinical features according to the 2016 WHO classification standard of the central nervous system tumors.Results Among the 398 cases,222 cases(55.8%)were located on the supratentorial region,and 147 cases(36.9%)were in the infratentorial and 37 cases(9.3%)in the spinal canal.The average age of these children was 8.99 years,and the age distribution was:<=3 years(54 cases,13.6%),4 to 9 years(157 cases,39.8%),and 10 to 16 years(187 cases,47.0%);Male:female ratio was 1.1∶1.0.Pathological types of top five were:astrocytic tumor(88 cases,22.1%),medulloblastoma(86 cases,21.6%),craniopharyngioma(51 cases,12.8%),ependymal tumor(42 cases,10.6%),and germ cell tumor(19 cases,4.8%).Conclusion In the central nervous system tumors of children in Xinjiang,supratentorial tumors incidence is higher than infratentorial,and the sex ratio is equal proportion.The histopathological types are related to the factors such as age,gender,and location.Understanding its characteristics plays an important role in the correct pathological diagnosis and clinical treatment.