伴有间脑综合征的儿童视路胶质瘤的临床治疗效果

Treatment outcome of optic pathway glioma in children with diencephalon syndrome

目的探讨伴有间脑综合征的视路胶质瘤的临床治疗效果。方法回顾性分析2015年7月至2018年12月首都医科大学附属北京儿童医院神经外科收治的8例伴有间脑综合征的视路胶质瘤患儿的临床资料。患儿就诊年龄为8.0~42.0个月;均在1岁前出现体重不增的现象,且入院时患儿已出现极度消瘦的恶病质状态。8例患儿就诊时的体重为5.5~11.5 kg,其年龄别体重Z评分均<-2。8例视路胶质瘤均行显微外科手术治疗,并于术后开始化疗。结果8例伴有间脑综合征的视路胶质瘤患儿中,肿瘤大部分切除7例,近全切除1例,无手术死亡病例。术后病理学确诊为毛细胞型星形细胞瘤2例,毛细胞黏液样星形细胞瘤6例。8例患儿的随访时间为1~18个月,术后体重为8.0~20.0 kg,均有不同程度的增长,同时部分患儿的临床症状得到缓解。1例患儿在随访期内死亡,7例经化疗后,4例残余肿瘤缩小,2例残余肿瘤增大,1例肿瘤在颅内播散转移。结论伴有间脑综合征的视路胶质瘤患儿经肿瘤大部切除术后消瘦症状明显改善,部分症状能够缓解,但肿瘤对术后化疗的敏感性具有较大差异。

Objective To explore the clinical treatment outcomes of the optic pathway glioma in children with diencephalic syndrome.Methods The clinical data of 8 cases of optic pathway glioma with diencephalic syndrome were analyzed retrospectively who were admitted to Department of Neurosurgery,Beijing Children′s Hospital,Capital Medical University from July 2015 to December 2018.The range of the children′s ages was from 8.0 to 42.2 months.No weight gain was reported in all those patients before the age of 1 year,and they showed a cachexia state of extreme emaciation at admission.The range of children′s preoperative weights was from 5.5 to 11.5 kg.The weight-for-age Z score was lower than-2 in all 8 patients who underwent microsurgical resection of tumor followed by chemotherapy post operation.Results Among the 8 cases of optic pathway glioma combined with diencephalic syndrome,subtotal resection was achieved in 7 cases,near-total resection in 1,and there was no death related to operation.Postoperative pathological examination confirmed 2 cases of pilocytic astrocytoma and 6 cases of pilomyxoid astrocytoma.The range of follow-up period of 8 children was from 1 to 18 months,the range of postoperative weight was from 8.0 to 20.0 kg,the weight of all patients increased to various degrees,and some of the patients′clinical symptoms were alleviated.One patient died after discharge,and chemotherapy was applied to the rest of 7 patients.Residual tumor shrank in 4 patients,progressed in 2 and disseminated in 1.Conclusions Subtotal tumor resection in children with optic pathway gliomas and diencephalic syndrome could significantly improve the symptom of emaciation and alleviate some other symptoms.There seems to be great variability in the tumor sensitivity to postoperative chemotherapy.