儿童弥散内生型脑桥胶质瘤患者的非典型临床表现及其预后

Atypical clinical manifestations and prognosis analysis of pediatric diffuse intrinsic pontine glioma

目的分析儿童弥散内生型脑桥胶质瘤患者的非典型临床表现及其预后。方法回顾性分析2015年11月至2017年11月首都医科大学附属北京天坛医院神经外科收治的27例儿童弥散内生型脑桥胶质瘤患者的临床资料。根据患儿是否存在非典型临床表现分为非典型组(17例)和对照组(10例)。27例患儿中,16例采用立体定向或开颅活组织检查术,11例采用手术切除治疗。术后对所有患儿行门诊或电话随访,询问患儿的临床转归。比较两组患儿的临床资料,进一步采用Kaplan-Meier生存曲线比较两组患儿的预后。结果27例患儿的病理学检查结果为,20例为高级别胶质瘤,其中4例为弥漫性中线胶质瘤、6例为胶质母细胞瘤、6例为间变性少突星形细胞瘤、4例为间变性星形细胞瘤;7例为低级别胶质瘤,其中4例为少突星形细胞瘤、3例为星形细胞瘤。开颅术后1例患儿发生肺部感染,治疗后痊愈出院。非典型组和对照组患儿的年龄、性别、临床表现(包括脑神经功能障碍表现、长束征、小脑征)、影像学表现(包括有无强化病灶、小脑幕上脑室扩大及脑桥臂受累)、治疗方法及病理学方面的差异均无统计学意义(均P>0.05)。Kaplan-Meier生存曲线显示,非典型组患儿的累积生存率明显高于对照组(P=0.045)。结论部分儿童弥散内生型脑桥胶质瘤患者在出现典型临床症状前有非典型临床表现,且出现可能提示患儿的预后更好。

Objective To analyze the atypical clinical manifestations and clinical prognosis of pediatric diffuse intrinsic pontine glioma(DIPG).Methods A total of 27 patients with complete clinical data of DIPG undergoing clinical treatment at Neurosurgery Department of Beijing Tiantan Hospital,Capital Medical University from November 2015 to November 2017 were retrospectively enrolled into this study.All patients were divided into 2 groups according to the presence of atypical manifestations:atypical clinical manifestation group(17 cases)and the control group(10 cases).Among the 27 cases,16 patients underwent stereotactic biopsy or open cranial biopsy,and the other 11 patients underwent tumor excision through craniotomy.All the children were followed up clinically or by telephone after operation to study the clinical outcomes.Comparative analysis was conducted on those clinical data.Prognosis was compared between the 2 groups through Kaplan-Meier survival analysis.Results Among the 27 cases,20 were pathologically identified as high grade gliomas(including 4 cases of diffuse midline glioma,6 cases of glioblastoma,6 cases of anaplastic oligoastrocytoma,4 cases of anaplastic astrocytoma)and 7 as low grade glioma(including 4 cases of oligoastrocytoma and 3 cases of astrocytoma).One child suffered from pneumonia and finally recovered postoperatively.No statistically significant difference(all P>0.05)was identified between 2 groups in the age,gender,clinical manifestation(including cranial nerve palsy,long tract dysfunction and cerebellum signs),clinical imaging features(including lesion enhancement,supratentorial ventricle enlargement and brachium pontis involvement),clinical treatment or pathology.Kaplan-Meier survival analysis showed that patients with atypical clinical manifestation had significantly higher cumulative survival rate than those in the control group(P=0.045).Conclusion Atypical clinical manifestation could be identified in some pediatric DIPG patients,which might be predictive of a better prognosis.