儿童脑干胶质瘤的临床诊治及预后分析

Clinical diagnosis,treatment and prognosis analysis of brainstem glioma in children

目的:探讨儿童脑干胶质瘤(BSG)的临床特点、诊疗措施及预后。方法:回顾性分析2015年1月至2020年1月清华大学玉泉医院神经外科中心行手术治疗的32例(年龄<18岁)BSG患儿的临床资料。肿瘤位于中脑9例,脑桥19例,延髓4例。根据肿瘤的发生部位、生长特点以及肿瘤与神经纤维束的关系等分别采用经枕下后正中入路(14例)、经乙状窦后入路(6例)、经小脑水平裂入路(4例)、经枕下小脑幕上入路(2例)、经小脑幕上下联合入路(1例)、经胼胝体-穹窿间入路(3例)和经颞下入路(2例);术中均行神经电生理监测。术后根据肿瘤的切除程度及病理学分级确定是否行放化疗,并定期行临床及影像学随访。结果:32例BSG患儿的男女比例为2.2∶1.0,发病年龄为(6.8±3.4)岁(1~17岁)。其中脑神经麻痹19例,脑积水13例,颈部僵硬或斜颈8例,Parinaud综合征3例。肿瘤的生长特点:外生型14例,局灶内生型5例,弥漫内生型11例,特殊类型2例。肿瘤全切除17例,次全切除4例,部分切除11例。术后病理学结果:世界卫生组织(WHO)分级Ⅰ级9例,Ⅱ级5例,Ⅲ级6例,Ⅳ级12例。术后共18例患儿给予辅助放化疗。32例患儿的中位随访时间为25个月(3~60个月),其中18例在随访期内因颅脑原发病死亡。16例内生型胶质瘤患儿均死亡,其中位生存期为7.5个月;14例外生型患儿死亡2例;2例特殊类型的患儿均存活。14例WHOⅠ~Ⅱ级的患儿死亡2例,其余患儿至末次随访时均存活且无复发;18例WHOⅢ~Ⅳ级的患儿死亡16例,其中位生存期为11.5个月。结论:儿童BSG患儿的临床表现以脑神经麻痹和脑积水居多,影像及病理学特点多样;治疗方案包括手术及放化疗;外生型、特殊类型及低级别BSG患儿的预后良好,内生型及高级别BSG患儿的预后较差。

Objective To investigate the clinical features,diagnosis,treatment and prognosis of brain stem gliomas(BSG)in children.Methods The clinical data of 32 patients(<18 years old)with BSG who underwent surgery at Neurosurgery Center of Tsinghua University Yuquan Hospital from January 2015 to January 2020 were retrospectively analyzed.Among them,9 cases originated from the midbrain,19 from the pons and 4 from the medulla oblongata.According to the tumor location,growth characteristics and the relationship between nerve fiber tracts and tumors,the suboccipital posterior median approach was taken in 14 cases,retrosigmoid sinus approach in 6 cases,cerebellar horizontal fissure approach in 4 cases,suboccipital supratentorial approach in 2 cases,supratentorial combined approach in 1 case,transcallosal interforniceal approach in 3 cases and subtemporal approach in 2 cases.Intraoperative electrophysiological monitoring was performed during all operations.After surgery,whether to undergo radiotherapy and chemotherapy was determined according to the degree of tumor resection and pathological grade.Regular clinical and imaging follow-up was performed.Results The male to female ratio of 32 patients with BSG was 2.2∶1.0,and the age of onset was 6.8±3.4(1-17)years old.There were 19 cases of cranial nerve palsy,13 cases of hydrocephalus,8 cases of stiff neck or torticollis,and 3 cases of Parinaud syndrome.According to the characteristics of tumor growth,there were 14 cases of exophytic type,5 cases of focal endophytic type,11 cases of diffuse endophytic type and 2 cases of special type.Total resection was achieved in 17 cases,subtotal resection in 4 cases and partial resection in 11 cases.Postoperative pathological results revealed that there were 9 cases of gradeⅠ,5 cases of gradeⅡ,6 cases of gradeⅢand 12 cases of gradeⅣaccording to WHO classification.A total of 18 patients were treated with adjuvant chemoradiotherapy.The median follow-up time of 32 patients was 25 months(3-60 months).Among them,18 patients died of primary brain disease during the follow-up period,with the median survival time of 7.5 months.All 6 patients with BSG of endophytic type died,with a median survival time of 7.5 months;2 out of 14 patients with exophytic type died and 2 patients with special type survived.Among the 14 patients with tumors of WHO gradeⅠ-Ⅱ,2 died,and the rest survived without recurrence at the last follow-up.Among the 18 patients with tumors of gradeⅢ-Ⅳ,16 died,with a median survival of 11.5 months.Conclusions The clinical manifestations of children with BSG are mainly cranial nerve palsy and hydrocephalus,with various imaging and pathological features.The treatment options include surgery,radiotherapy and chemotherapy.The prognosis of exophytic type,special type or low-grade BSG seems relatively favorable,while the that of endophytic type or high-grade BSG is relatively poor.