儿童非典型脉络丛乳头状瘤的临床特点及疗效观察  

Clinical characteristics and therapeutic effect of atypical choroid plexus papilloma in children

作　　者：赵凤毛[1] 葛明[1] 孙骇浪[1] 张楠[1] Zhao Fengmao;Ge Ming;Sun Hailang;Zhang Nan(Department of Neurosurgery,Beijing Children′s Hospital,Capital Medical University,National Children′s Medical Center,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院神经外科,北京100045

出　　处：《中华神经外科杂志》2022年第9期895-899,共5页Chinese Journal of Neurosurgery

摘　　要：目的探讨儿童非典型脉络丛乳头状瘤(aCPP)的临床特点、治疗方法及其预后。方法回顾性分析2014年1月至2021年11月首都医科大学附属北京儿童医院神经外科收治的10例aCPP患儿的临床资料。其中,7例肿瘤仅累及一侧侧脑室及其三角区,行单侧三角区入路肿瘤切除术;另2例累及第三脑室及双侧侧脑室,1例累及颞、顶、枕叶,行胼胝体-穹窿间入路肿瘤切除术+硬膜下或脑室外引流术,后期行硬膜下-腹腔或脑室-腹腔分流手术。术后1、3、6个月,1年以及之后每年对患儿进行门诊或电话随访。随访术后是否行放化疗、二次手术治疗,有无新发神经功能障碍等。影像学随访有无肿瘤播散、转移。以术后头颅MRI增强扫描显示颅内瘤腔未见强化为肿瘤全切除。结果10例患儿的中位就诊年龄为1岁(3个月至11岁2个月),其中8例年龄<2岁。所有患儿的病变均位于小脑幕上,均达肿瘤全切除,术后症状改善。10例患儿的病理学分级均为世界卫生组织(WHO)Ⅱ级;免疫组织化学染色结果显示,波形蛋白均为阳性,细胞角蛋白(CK)和S-100均为弱阳性,Ki-67增殖指数为6%~30%。10例患儿的术后中位随访时间为51个月(6个月至8年)。9例患儿术后未行化疗,1例术后2年因急性淋巴细胞白血病行相关化疗。至末次随访,3例后期行分流手术的患儿均未出现分流相关并发症,且肌力均较前进一步改善;另7例患儿中,1例术前合并颅脑外伤的患儿死亡,余6例症状缓解。9例存活患儿的头颅增强MRI结果显示,颅内瘤腔未见强化,无一例发生脑脊液播散、转移或复发。结论儿童aCPP多于2岁前发病,多起源于小脑幕上脑室内脉络丛。经开颅手术治疗,肿瘤可达全切除,总体预后较好。Objective To investigate the clinical characteristics,treatment methods and prognosis of atypical choroid plexus papilloma(aCPP)in children.Methods The clinical data of 10 children with aCPP admitted to the Department of Neurosurgery,Beijing Children′s Hospital,Capital Medical University from January 2014 to November 2021 were retrospectively analyzed.Among them,7 cases of tumors involved only one side of the lateral ventricle and its trigone region,and tumor resection was performed through unilateral trigone region approach.For the other 2 cases involved the third ventricle and bilateral lateral ventricles,and 1 case involved the temporal,parietal,and occipital lobes,tumor resection via interfornical approach of corpus callosum plus subdural or extraventricular drainage were performed,and subdural-peritoneal or ventriculoperitoneal shunt was performed later.Outpatient or telephone follow-up was performed at 1,3,and 6 months after surgery,1 year after surgery,and annually thereafter.Postoperative radiotherapy and chemotherapy,secondary surgical treatment,new-onset neurological dysfunction,etc.were followed up.Imaging follow-up was performed for tumor spread and metastasis.No enhancement in the intracranial tumor cavity on postoperative brain MRI enhanced scan indicated total tumor resection.Results The median age at presentation of the 10 patients was 1 year(3 months to 11 years and 2 months),and 8 of them were younger than 2 years.All patients lesions were supratentorial and achieved total tumor resection,and postoperative symptoms improved.The pathological grades of 10 children were all World Health Organization(WHO)gradeⅡ;immunohistochemical staining showed that all of them were positive for vimentin,weakly positive for cytokeratin(CK)and S-100,and the Ki-67 index was 6%to 30%.The median postoperative follow-up of the 10 patients was 51 months(6 months to 8 years).Nine cases did not receive chemotherapy after operation,and one case received related chemotherapy for acute lymphoblastic leukemia 2 years afte

关 键 词：脉络丛肿瘤 乳头状瘤 儿童 疾病特征 治疗结果 

分 类 号：R739.41[医药卫生—肿瘤]