双侧丘脑胶质瘤临床特点、治疗及预后分析  

Clinical characteristics,treatment and prognosis of bilateral thalamic gliomas

作　　者：杨帆[2] 韩铖琛 董超[1] 程岗 张剑宁 Yang Fan;Han Chengchen;Dong Chao;Cheng Gang;Zhang Jianning(Department of Neurosurgery,the Sixth Medical Center of PLA General Hospital,Beijing 100048,China;Department of Neurosurgery,the First Medical Center of PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第六医学中心神经外科,北京100048 [2]解放军总医院第一医学中心神经外科,北京100853

出　　处：《中华神经外科杂志》2021年第6期577-581,共5页Chinese Journal of Neurosurgery

基　　金：解放军总医院第六医学中心创新培育基金(CXPY201908)。

摘　　要：目的探讨双侧丘脑胶质瘤的临床特点、治疗及预后。方法回顾性分析2015年1月至2019年12月解放军总医院第六医学中心神经外科通过立体定向活组织检查术明确病理诊断的18例双侧丘脑胶质瘤患者的临床资料。以门诊复查和电话回访的方式随访患者的后续治疗生存状况及生存期。结果18例患者的中位年龄为35.5岁(3岁4个月至68岁),成人15例,儿童3例;男、女患者的比例为1.25:1.00。首发症状表现为颅内压增高症状者4例,嗜睡3例,复视3例,癫痫发作3例,眼睑下垂2例,听力下降1例,肢体无力1例,流涎1例。18例患者均明确病理诊断,其中异柠檬酸脱氢酶(IDH)野生型弥漫性星形细胞瘤10例,IDH野生型间变性星形细胞瘤2例,H3K27M突变型弥漫性中线胶质瘤6例;13例存在表皮生长因子受体阳性,Ki-67标记指数为3%-20%,无一例发生IDHI和ax地中海贫血伴智力低下综合征X连锁(ATRX)基因突变。18例患者术后的中位随访时间为10个月(6周至21个月)。明确诊断后行放疗1例,替莫唑胺化疗2例,同步放化疗15例;9例肿瘤继发脑积水,其中5例患者行脑室-腹腔分流术。至末次随访,16例患者死亡,2例存活,16例患者的中位生存期为13个月(6周至21个月)。结论双侧丘脑胶质瘤常见于成人,临床症状多不典型,且多继发脑积水,明确诊断后可给予放化疗及脑室-腹腔分流术等综合治疗,但该病总体预后较差。Objective To discuss the clinical characteristics,treatment and prognosis of bilateral thalamic gliomas.Methods A retrospective analysis was conducted on the clinical data of 18 patients who were diagnosed with bilateral thalamic gliomas based on stereotactic biopsy from January 2015 to December 2019 at Department of Neurosurgery,the Sixth Medical Center of PLA General Hospital.The patientfs subsequent therapy,survival status and overall survival were followed up through outpatient reexamination and telephone inquiry.Results The median age of the 18 patients(15 adults and 3 children)was 35.5 years(range:3 years and 4 months to 68 years),and the male/female ratio was 1.25:1.00.The initial symptoms were intracranial hypertension in 4 cases,somnolence in 3 cases,diplopia in 3 cases,epileptic seizure in 3 cases,blepharoptosis in 2 cases,hearing impairment in 1 case,limb weakness in 1 case,and salivation in 1 case.Pathological diagnoses were established in all 18 cases including IDH(isocitrate dehydrogenase)wild type diffuse astrocytoma in 10 cases,IDH wild type anaplastic astrocytoma in 2 cases,and H3 K27M mutant diffuse midline glioma in 6 cases.Epidermal growth factor receptor was positive in 13 patients,and the Ki-67 index ranged from 3%to 20%.None of patients had IDH1 mutation or X-linked alpha-thalassemia/mental retardation(ATRX)mutation.The median postoperative follow-up period for the 18 patients was 10 months(range:6 weeks to 21 months).Among those 18 patients,radiotherapy was performed in 1 case,temozolomide chemotherapy in 2 cases,and chemoradiotherapy in 15 cases following biopsy-based diagnosis.Nine patients developed secondary hydrocephalus and 5 out of them underwent ventriculoperitoneal shunting.At the latest follow-up,there were 16 deaths and 2 survivors.The median survival period of 16 patients was 13 months(range:6 weeks to 21 months).Conclusions Bilateral thalamic gliomas,commonly seen in adults,are often associated with atypical clinical symptoms and secondary hydrocephalus.Comprehensive treatment

关 键 词：神经胶质瘤 疾病特征 病理学 分子 预后 双侧丘脑 

分 类 号：R739.41[医药卫生—肿瘤]