儿童幕上室管膜瘤（附19例临床分析）

１９８２～１９９３年收治１９例儿童幕上室管膜瘤。其中，男１２例，女７例，年龄９个月～１４岁。幕上室管膜瘤少见，大多位于脑实质内，病程比幕下室管膜瘤长。ＣＴ扫描显示较高的钙化率及囊变率。该病术后复发率高，本组为２１．１％（４例）。术后放疗可有效延缓或防止肿瘤复发，预防性脊髓放射并非常规。肿瘤复发是影响预后的主要因素。     

儿童幕上室管膜瘤

１９８２～１９９３年收治１９例儿童幕上室管膜瘤。其中，男１２例，女７例，年龄９个月～１４岁。幕上室管膜瘤少见，大多位于脑实质内，病程比幕下室管膜瘤长。ＣＴ扫描显示较高的钙比率及囊变率。该病术后复发率高，本组为２１．１％（４例）。术后放疗可用效延缓或防止肿瘤复发，预防性脊髓放射并非常规。肿瘤复发是影响预后的主要因素。     

儿童第三脑室肿瘤显微手术治疗临床分析

目的 探讨显微手术切除儿童第三脑室肿瘤的手术方法 与术后管理.方法 回顾性分析2005年6月至2008年6月全组17例儿童第三脑室肿瘤患儿的临床资料,其中男10例,女7例.年龄1～14岁,平均8.3岁.病程1～33个月,平均9个月,经术前评估,选择经胼胝体-穹窿间人路切除肿瘤12例、经纵裂-经终板入路显微镜下手术切除肿瘤5例.术后病理证实颅咽管瘤9例,畸胎瘤4例,生殖细胞瘤3例,星形细胞瘤1例.结果 17例第三脑室肿瘤患儿,肿瘤全切除8例(47.1%),近全切除7例(41.2%),大部切除2例(11.7%),无死亡.术后11例出现下丘脑损害并发症.结论 正确的术前评估、选择合适的手术入路、重视术后管理是手术治疗儿童第三脑室肿瘤获得良好效果的重要环节.经胼胝体-穹窿间入路术野暴露清楚,较易全切肿瘤,术后并发症少,是切除第三脑室肿瘤值得选择的手术入路.     

儿童第三脑室肿瘤显微手术治疗临床分析

目的 探讨显微手术切除儿童第三脑室肿瘤的手术方法 与术后管理.方法 回顾性分析2005年6月至2008年6月全组17例儿童第三脑室肿瘤患儿的临床资料,其中男10例,女7例.年龄1～14岁,平均8.3岁.病程1～33个月,平均9个月,经术前评估,选择经胼胝体-穹窿间人路切除肿瘤12例、经纵裂-经终板入路显微镜下手术切除肿瘤5例.术后病理证实颅咽管瘤9例,畸胎瘤4例,生殖细胞瘤3例,星形细胞瘤1例.结果 17例第三脑室肿瘤患儿,肿瘤全切除8例(47.1%),近全切除7例(41.2%),大部切除2例(11.7%),无死亡.术后11例出现下丘脑损害并发症.结论 正确的术前评估、选择合适的手术入路、重视术后管理是手术治疗儿童第三脑室肿瘤获得良好效果的重要环节.经胼胝体-穹窿间入路术野暴露清楚,较易全切肿瘤,术后并发症少,是切除第三脑室肿瘤值得选择的手术入路.     

儿童第三脑室肿瘤显微手术治疗临床分析

目的 探讨显微手术切除儿童第三脑室肿瘤的手术方法 与术后管理.方法 回顾性分析2005年6月至2008年6月全组17例儿童第三脑室肿瘤患儿的临床资料,其中男10例,女7例.年龄1～14岁,平均8.3岁.病程1～33个月,平均9个月,经术前评估,选择经胼胝体-穹窿间人路切除肿瘤12例、经纵裂-经终板入路显微镜下手术切除肿瘤5例.术后病理证实颅咽管瘤9例,畸胎瘤4例,生殖细胞瘤3例,星形细胞瘤1例.结果 17例第三脑室肿瘤患儿,肿瘤全切除8例(47.1%),近全切除7例(41.2%),大部切除2例(11.7%),无死亡.术后11例出现下丘脑损害并发症.结论 正确的术前评估、选择合适的手术入路、重视术后管理是手术治疗儿童第三脑室肿瘤获得良好效果的重要环节.经胼胝体-穹窿间入路术野暴露清楚,较易全切肿瘤,术后并发症少,是切除第三脑室肿瘤值得选择的手术入路.     

儿童颅内室管膜瘤临床特点及预后因素分析

目的探讨儿童颅内室管膜瘤的临床特点、诊疗分析及其预后影响因素。方法回顾性分析2012年1月至2020年12月首都医科大学附属北京儿童医院神经外科收治的32例儿童颅内室管膜瘤患儿的临床资料。男23例, 女9例;诊断中位年龄为4.5岁(0.7～10.0岁)。其中<3岁者12例(37.5%), ≥3岁者20例(62.5%)。肿瘤位于幕上14例, 幕下18例。患儿均行肿瘤切除术, 术后放疗18例, 术后化疗20例, 未行放疗及化疗4例。采用Kaplan-Meier法绘制颅内室管膜瘤患儿的生存曲线;采用Log-rank检验单一变量差异是否有统计学意义。结果 32例患儿中, 30例为全切除, 2例为未全切除。平均随访时间为24个月(1～62个月), 至随访截止存活26例, 死亡6例。1年生存率为88%, 3年为生存率为78%。单因素结果分析提示肿瘤部位(OR=1.5, 95%CI:1.082～2.079, P=0.040)对于术后生存时间差异有统计学意义, 是否进行化疗(OR=0.5, 95%CI:0.125～1.999, P=0.034)对于年龄<3岁患儿术后生存时间差异有统计学意义...     

儿童脑肿瘤的临床治疗及病理特点研究

目的探讨儿童脑肿瘤的诊疗、手术、病理特点及治疗效果。方法对1999年1月～2005年5月间我科手术的儿童脑肿瘤72例进行回顾性分析。结果恶性肿瘤51例，良性肿瘤21例，恶性肿瘤占70．8％；幕上肿瘤38例，其中恶性肿瘤20例。幕下肿瘤34例，其中恶性肿瘤31例。肿瘤全切除、次全切除49例，大部分切除13例，部分切除10例。术后放射治疗43例，占手术病例的59．7％。化疗26例，占手术病例的36．1％。随访到47例，其中恶性肿瘤仍存活21例，已死亡13例。结论儿童脑肿瘤的治疗以手术切除为主，根据各个病例的特殊性，制定不同的手术切除方案，术后进行放疗，但应有别于成人脑肿瘤。恶性肿瘤选择适合的病例进行化疗，对于延长儿童的生存期是有效的。     

儿童髓母细胞瘤显微手术治疗

目的：探讨儿童髓母细胞瘤的显微手术方法及相关问题。方法：回顾性分析经四脑室顶部切开联合四脑室正中孔入路显微手术切除并经病理证实的小儿髓母细胞瘤27例。结果：手术全切除22例(81.5%)、次全切5例(18.5%)。术后26例颅高压症状消失，临床体征改善，1例恶化，无与手术有关的严重并发症和手术死亡。肿瘤位于小脑蚓部6例，四脑室内9例，小脑蚓部和四脑室内者11例，小脑半球1例。肿瘤供血来源于双侧小脑后下动脉分支19例。术中打通导水管26例，1例术中同时行枕大池分流。术后25例病人随访5个月至3年4个月，并接受放疗。其中23例未见复发或转移，1例转移至骨髓，3例原位复发。结论：正确认识小儿髓母细胞瘤的病理解剖结构及其周围组织结构有利于肿瘤切除，采用显微手术全切除肿瘤组织, 妥善保护好小脑后下动脉及分支，避免伤及脑干和四脑室底的结构，术中解除导水管梗阻，是降低复发和转移率、延长生存期的关键。     

显微技术切除儿童侧脑室肿瘤

目的：探讨儿童侧脑室肿瘤显微外科手术的治疗方式和临床效果。方法：回顾性总结51例儿童侧脑室肿瘤的临床表现、诊断方式、手术入路的选择与疗效。根据肿瘤的部位和大小分别采用经额中回入路、经顶枕叶或顶上小叶入路、经胼胝体入路，对儿童侧脑室肿瘤进行显微外科手术治疗。结果：51例儿童侧脑室肿瘤中，位于侧脑室三角区27例，侧脑室前角12例，侧脑室体部9例，室间孔区3例，左侧脑室27例，右侧脑室24例。肿瘤直径2～6．5cm。所有肿瘤均经手术和病理证实，肿瘤全切除43例，近全切除6例，大部切除1例，部分切除加外减压1例。31例获得随访，28例生活基本上能自理或参加学习。结论对儿童侧脑室肿瘤选择适当的手术入路进行显微外科手术能有效地降低手术创伤，提高全切除率，减少致残率和病死率，对于无法手术切除的肿瘤可以采用术后放疗和立体定向放射外科治疗，可减少本病复发。     

手术完全切除肿瘤对晚期神经母细胞瘤患儿生存率的影响

目的分析21例Ⅲ期、Ⅳ期腹膜后神经母细胞瘤患儿经血管骨骼化方法完全切除肿瘤后的临床疗效,探讨手术完全切除肿瘤对晚期神经母细胞瘤患儿生存率的影响。方法2003年至2008年,对本院收治的22例临床诊断为Ⅲ期、Ⅳ期神经母细胞瘤的患儿,经穿刺活检病理诊断明确为神经母细胞瘤后,按照最新国际标准化疗方案行4～6个疗程的化疗,待肿瘤体积缩小后,采用腹膜后大血管骨骼化的方法实施手术,21例完全切除肿瘤,并继续化疗等治疗。通过门诊复查及电话、信件随访的方式了解术后康复情况,包括有无原位复发、有无远隔转移、是否存活以及死亡原因等。结果21例延期一期手术完全切除肿瘤（占95．45％）,无严重的术后并发症及围手术期死亡。失访3例。随访病例中,无瘤存活10例,占55．56％;带瘤存活3例,占16．67％;死亡5例,占27．78％,5年总体生存率为72．22％（13／18）。结论通过腹膜后大血管骨骼化方法完全切除儿童Ⅲ期、Ⅳ期神经母细胞瘤,能显著提高术后生存率。     

Intracranial ependymoma in children. Prognosis and therapeutic perspectives

The authors report 47 cases of intracranial ependymomas occurring in children less than 15 years of age, who were operated on from 1969 to 1979. The aim of the study was to study the incidence of intraspinal metastases and to suggest a logical protocol for postoperative radiotherapy. The operative mortality rate was 17%. The 5 year survival rate, after exclusion of postoperative mortality, was 51%. Recurrences developed in 41% of cases and metastases in 20%. Among these, intraspinal metastases occurred in 14.5% and were found only in infratentorial ependymomas, whether benign or malignant. The results and those in the literature suggest that a protocol of irradiation adapted to the site and the histological grading of these tumors should be discussed with the teams of radiotherapy; in infratentorial ependymomas, the irradiation should include the whole craniospinal axis.     

Brain tumors in children under 3 years of age. Recent experience (1987-1997) in 39 patients.

Brain tumors in children under 3 years of age differ in clinical presentation and pathological behavior from those in older patients. In this study, we reviewed data from 39 children (24 males and 15 females) under 3 years of age who were treated for intracranial brain tumors since the introduction of magnetic resonance imaging. The purpose was to assess correlations between clinicoradiological and treatment-related factors, and their impact on prognosis. The following factors were analyzed: sex, age, duration of symptoms, intracranial hypertension on admission, tumor location, surgical removal and histology. Associations between these factors and correlations with prognosis were determined using bivariate analyses (chi(2) test) and Kaplan-Meier survival curves. Collins' concept of a period of risk recurrence was tested. Mean follow-up was 41 months (range 0-136). In March 1998, 20 children were still alive (51.2%) with a mean survival time of 65 months (range 2-136). The incidence of supratentorial tumors was significantly higher in children less than 1 year old (p = 0.027). Lateral tumors were 9/10 (90%) supratentorial versus only 7/26 (27%) midline tumors (p = 0.001). Outcome (dead or alive) was significantly better (p = 0.037) for low-grade astrocytomas (9/12 = 75% survival) when compared to ependymomas (2/6 = 33%) and primitive neuroectodermal tumors (3/12 = 25%). Total tumor removal was achieved in 20 cases and was associated with a better outcome (65 vs. 33% survival; p = 0.049). Survival analysis confirmed a worse prognosis for children with ependymomas and primitive neuroectodermal tumors (p = 0.011) and revealed a worse survival for children with intracranial hypertension on admission (p = 0.047). Total tumor removal was associated with a longer survival, although not significantly (p = 0. 077). Finally, we found no exceptions to Collins' law.     

儿童囊性毛细胞型星形细胞瘤的治疗

目的 总结儿童囊性毛细胞型星形细胞瘤的临床特点和治疗经验。方法 回顾性分析过去5年间我科收治的20例儿童囊性毛细胞型星形细胞瘤的临床资料。本组20例，占同期住院胶质瘤的6．0％，发病年龄平均为9岁。病变在小脑者14例，下视丘/视路2例，三脑室内2例，大脑半球内2例。20例患儿均接受显微手术治疗。肿瘤位于小脑幕者采用下正中入路；肿瘤位于下视丘／视路者采用翼点入路；三脑室内肿瘤选择胼胝体-透明隔入路，颞叶肿瘤均采用额颞瓣开颅。肿瘤全切除18例。2例术后有残余者接受放射治疗。照射剂量30～40Gy。无手术死亡。结果 术后1周内行影像学检查以证实切除程度。20例在术后24h行头颅CT检查，12例在术后4～6周行MRI检查做为以后MRI检查的对比基础。18例随访半年以上无复发。结论 儿童囊性毛细胞型星形细胞瘤为良性肿瘤，预后较好，显微手术切除和恰当的放疗是治疗的关键。     

Intracranial ependymomas of childhood: current management strategies

Children with intracranial ependymomas have relatively poor outcomes despite the low-grade histology of these tumors and recent advances in diagnosis, microneurosurgical resection, and adjuvant therapy. Aggressive surgical resection and postoperative adjuvant therapy result in only a 5-year survival rate of 50%. In this paper, we provide a review of the current technical aspects of surgical resection, and focus on recent studies of the epidemiology, molecular markers, prognostic factors and adjuvant therapy for childhood intracranial ependymomas and discuss their implications for current and future management strategies.     

Descriptive epidemiology of pediatric intracranial neoplasms in egypt

Objective: The characteristics of 451 Egyptian children (aged 0-14 years) with primary intracranial neoplasms were investigated for demographic, clinical, topographical and pathological features using the most recent 2007 Classification of Central Nervous System Tumors. Patients and Methods: This was a retrospective study performed in the Departments of Pediatric Neurosurgery of the Cairo University Hospitals from 2005 to 2008. Results: There was a slight male predominance (51.4%) observed in our study, and the most affected age group was 5-9 years old (43.2%). Most of the tumors were confined to a single compartment (infratentorial in 49.7%, supratentorial in 46.6%), while 3.8% of the tumors involved multiple compartments. The most common intracranial tumors were astrocytomas (35%), medulloblastomas (18.8%), craniopharyngiomas (11.3%) and ependymomas (10%). Pilocytic astrocytomas constituted 55% of all astrocytomas and 19.3% of all brain tumors, only slightly ahead of medulloblastomas. Less common types were primitive neuroectodermal tumors (2.7%), followed by meningiomas, germ cell tumors and choroid plexus tumors (2.4% each). According to the International Classification of Diseases for Oncology Coding (ICD-O-4), benign, borderline and malignant tumors constituted 7.54, 36.14 and 56.32%, respectively. Conclusion: The characteristics of pediatric intracranial tumors in Egypt are generally similar to those reported in the literature, with only minor differences.     

Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone

BACKGROUND: To determine the effectiveness of multiagent chemotherapy as sole post-operative treatment of supratentorial central nervous system (CNS) embryonal tumors in young children. PROCEDURE: The data of 25 children under 5 years of age diagnosed with supratentorial embryonal tumors (17 primitive neuroectodermal tumors, four pinealoblastomas, and four medulloepitheliomas) treated exclusively by postoperative chemotherapy (CT) between 1990 and 1997 were reviewed. RESULTS: Fifteen tumors were hemispheric and 10 were deeply seated. Four children presented with disseminated leptomeningeal disease. Total resection was performed in nine patients, subtotal in 9, partial in 3, and a diagnostic biopsy only in 2. Two children did not undergo surgery. Twenty-four children relapsed with a median time of 5.5 months. The median overall survival was 12 months, and the 2-, and 5- year survivals were 30 and 14%, respectively. The 2- year disease-free survival was 4%. There was a significantly worse prognosis in patients undergoing incomplete resection and in the group with deeply situated tumors. Four relapses were treated by second surgery followed by high-dose chemotherapy and radiotherapy. Two of them remain in CR2, and all these children are free of late sequelae. CONCLUSIONS: CT alone failed to maintain disease-free survival in most of the children, although, disease progression was delayed to some extent. Children under 5 years with supratentorial embryonal tumors should undergo total surgical resection if possible.     

Prognostic factors in childhood ependymomas

Between 1975 and 1989, 45 children with newly diagnosed intracranial ependymomas were treated at the authors' institution. Patients were managed with aggressive surgical resection, followed by postoperative CT or MRI scans to evaluate the extent of resection. Most patients received involved-field radiation therapy, however 10 were treated with craniospinal axis irradiation for disseminated disease, or malignant histology. Beginning in 1983, all patients were also treated with adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatinum. Four patients died in the immediate postoperative period for an operative mortality of 8.8%. The 5-year progression-free survival for the remaining 41 patients was 36%. Tumor location had little effect on survival, as the 5-year PFS for supratentorial tumors was 26%, compared with 40% for the posterior fossa lesions (ns). Neither histologic degree of malignancy, nor use of adjuvant chemotherapy impacted on survival. The extent of surgical resection, as determined by operative reports and postoperative imaging studies, was a major determinant of outcome, as patients with total or near-total resections experienced a 5-year PFS of 60%, compared with 21% for children with partial resection or biopsy (p less than 0.01). It is concluded that the use of adjuvant chemotherapy with CCNU, vincristine, and cisplatinum does not improve progression-free survival in childhood ependymoma, and that the extent of surgical resection is a major determinant of outcome in this disease.     

Intracranial germ cell tumors in children with and without Down syndrome

PURPOSE: Two Chinese children with Down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. PATIENTS AND METHODS: Two children with Down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with Down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with Down syndrome.