经小脑延髓裂入路显微手术治疗儿童第四脑室肿瘤

目的 探讨经小脑延髓裂(CMF)人路显微手术治疗儿童第四脑室肿瘤的方法,提高手术治疗效果.方法 对17例2～14岁原发第四脑室肿瘤患儿,采用枕下正中切口、横窦下颅骨开窗骨瓣成形及经小脑延髓裂入路显微切除肿瘤,其中广泛型CMF切开12例,外侧壁型切开3例,外侧隐窝切开2例;硬脑膜严密缝合或修补,骨瓣复位并固定.结果 肿瘤全切除15例,次全切除2例,病理结果髓母细胞瘤9例,室管膜瘤5例,星形细胞瘤3例,无围手术期死亡发生.术后15例患儿症状体征均明显改善或消失,无新发共济失调、震颤、肌张力降低及脑积水、小脑缄默综合征等并发症.复发2例,均为髓母细胞瘤,2年后死亡1例.结论 经小脑延髓裂人路能较好地显露儿童第四脑室肿瘤,可减少因切开小脑蚓部和向侧方牵拉损伤小脑所导致的并发症发生.     

小脑延髓裂入路手术切除儿童第四脑室肿瘤

目的探讨经小脑延髓裂入路（ transcerebeliomedullochoroidal fissure, TCMCF）手术切除儿童第四脑室肿瘤的临床疗效。方法2004年11月至2010年11月,河南省开封市陇海（脑科）医院神经外科采用枕下正中切口,经小脑延髓裂入路,应用显微外科技术对20例第四脑室肿瘤患儿进行手术治疗。结果20例中,手术完全切除17例,次全切除3例;术后病理检查提示髓母细胞瘤12例,室管膜瘤6例,脉络丛乳头状瘤1例,星形细胞瘤1例。结论经小脑延髓裂入路,不需切开小脑蚓部,可避免损伤正常小脑组织,暴露范围广,有助于彻底切除第四脑室肿瘤,提高手术疗效。     

儿童后颅窝肿瘤的诊治特点

目的对儿童后颅窝肿瘤的临床特征、病理类型、手术切除及术后并发症进行分析。方法回顾分析我院儿童(15岁以下)后颅窝肿瘤30例，术前头颅CT及MRI确定肿瘤位置分别采用咬骨窗开颅(21例)和骨瓣开颅(9例)显微镜下切除肿瘤。结果全切除肿瘤20例，近全切除肿瘤10例。无手术死亡。术后病理证实：星形细胞瘤9例，髓母细胞瘤14例，室管膜瘤4例，恶性室管膜瘤3例。结论MRI能清楚地显示肿瘤的大小、部位、范围和界限，根据肿瘤不同部位选择最佳的手术入路，显微镜下暴露肿瘤最直视的部位分离肿瘤，可安全地全切除或近全切除肿瘤。     

儿童毛细胞黏液样星形细胞瘤的影像与病理特征研究

目的　探讨儿童毛细胞黏液样星形细胞瘤（PMA）的影像特征及其与病理学改变的关系。方法　回顾性分析2010年10月至2018年6月首都医科大学附属北京天坛医院收治的20例儿童颅内PMA患者的临床资料、CT和磁共振成像（MRI）影像表现以及病理和免疫组织化学结果。结果　20例儿童PMA患者中，男15例，女5例；肿瘤位于幕下小脑半球10例，幕上大脑半球5例，视交叉-下丘脑4例，三脑室内1例。在CT图像上，PMA表现为低密度10例；在MRI图像上，肿瘤呈混杂信号11例，T1WI低信号7例，T2WI高信号7例，DWI未见弥散受限12例，MRI增强扫描表现为不均匀强化17例；肿瘤边界清楚18例，发生囊变16例，瘤周水肿11例。组织病理学检查可见双极性梭形细胞，以血管为中心生长，间质内含有大量黏液，缺乏Rosenthal纤维。结论　儿童PMA具有较为特征性的影像表现和低度恶性的肿瘤特征，肿瘤不均质性、边界清楚、边缘囊变和不均匀强化是PMA的特征性表现，CT和MRI可为儿童PMA的术前诊断、临床治疗和预后评估提供参考。     

儿童毛细胞黏液样星形细胞瘤的影像与病理特征研究

目的　探讨儿童毛细胞黏液样星形细胞瘤（PMA）的影像特征及其与病理学改变的关系。方法　回顾性分析2010年10月至2018年6月首都医科大学附属北京天坛医院收治的20例儿童颅内PMA患者的临床资料、CT和磁共振成像（MRI）影像表现以及病理和免疫组织化学结果。结果　20例儿童PMA患者中,男15例,女5例;肿瘤位于幕下小脑半球10例,幕上大脑半球5例,视交叉-下丘脑4例,三脑室内1例。在CT图像上,PMA表现为低密度10例;在MRI图像上,肿瘤呈混杂信号11例,T1WI低信号7例,T2WI高信号7例,DWI未见弥散受限12例,MRI增强扫描表现为不均匀强化17例;肿瘤边界清楚18例,发生囊变16例,瘤周水肿11例。组织病理学检查可见双极性梭形细胞,以血管为中心生长,间质内含有大量黏液,缺乏Rosenthal纤维。结论　儿童PMA具有较为特征性的影像表现和低度恶性的肿瘤特征,肿瘤不均质性、边界清楚、边缘囊变和不均匀强化是PMA的特征性表现,CT和MRI可为儿童PMA的术前诊断、临床治疗和预后评估提供参考。     

毛细胞型星形细胞瘤的治疗

目的探讨毛细胞型星形细胞瘤的诊疗方法。方法 回顾性分析我科近10年来经病理诊断明确的11例该病患儿 11例中全切除9例,大部切除2例;术后放射治疗5例。结果 经1-10年随访,复发1例,11例均存活。结论毛细胞型星形细胞瘤属小儿颅内偏良性胶质瘤,若能达到手术全切除,可获良好结果。     

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。     

儿童颅内囊性病变的神经内镜手术治疗

目的 探讨儿童颅内囊性病变的神经内镜微侵袭手术治疗。方法 对6例小儿颅内囊性病变采用神经内镜手术，其中颅咽管并积水行囊壁大部切除 内镜下分流术，蛛网膜囊肿行囊壁部分切除 脑池交通术各2例，血管网织细胞瘤切除，畸胎瘤并积水行肿瘤部分切除 分流术各1例。结果 6倒均痊愈出院。MRI复查囊腔消失，并脑积水缓解。结论 神经内镜治疗小儿颅内囊性病变创伤小，手术简单，安全。     

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.     

Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1

Diencephalic gliomas may be grouped into 2 clinical categories. Optic pathway/hypothalamus gliomas (OPG) arise primarily from a slower-growing juvenile pilocytic astrocytoma, and thalamic gliomas arise primarily from a fibrillary astrocytoma which can become clinically and histologically more aggressive. Children with OPG have an excellent long-term prognosis with a 10-year survival of over 85%. The major therapeutic challenge for these patients is to maximize their quality of life by preserving visual and endocrine function while minimizing treatment-related morbidity. Treatment is often initiated at diagnosis in infants and toddlers who have a major visual impairment or the diencephalic syndrome. The judicious application of chemotherapy may serve to forestall the need for radiotherapy or surgery. Children with neurofibromatosis-1 (NF-1) usually have a more indolent course. Tumors may grow more slowly or occasionally regress spontaneously. However, over 90% of children with OPG without NF-1 will require some form of therapy. Patients with thalamic gliomas present with a shorter history, often with hydrocephalus. Surgical intervention is often required to relieve intracranial pressure and establish the histologic identity of the tumor. Over 75% of these tumors will become locally aggressive. Current multimodality therapy is relatively ineffective. The bithalamic variant behaves similarly to a pontine glioma.     

Prognostic factors for cerebellar astrocytomas in children: a study of 102 cases.

OBJECTIVE: To evaluate the prognostic factors influencing the length of survival of pediatric patients with cerebellar astrocytomas. METHODS: The clinical data of 102 patients under the age of 12 years with cerebellar astrocytomas were retrospectively analyzed. The radiological features, surgical findings, histology and adjuvant radiotherapy were reviewed. Patients were followed up on an outpatient basis. Computerized tomography (CT) scans of the brain were performed to detect recurrence. RESULTS: The age of the patients at presentation varied from 10 months to 12 years. The mean age at presentation was 7 years and 11 months. The average duration of symptoms was 5.8 months. The clinical features were predominantly related to intracranial hypertension and the location of the tumor. Twenty-six tumors were located in the vermis and 76 in the cerebellar hemisphere. The brain stem was involved in 20 patients. All 102 patients had a preoperative contrast-enhanced CT scan. Midline vermian tumors were predominantly solid and enhancing, whilst the hemispheric tumors were cystic and nonenhancing. The tumors were graded into three subgroups based on histological characteristics: pilocytic astrocytoma, low-grade fibrillary astrocytoma and high-grade fibrillary astrocytoma. Total, radical and subtotal excision, as determined by the surgical impression, were performed in 82, 16 and 4 patients, respectively. Postoperative radiotherapy was administered to 12 patients. CSF diversion was carried out in 11 of 79 cases who had moderate to severe hydrocephalus. CONCLUSIONS: Our study has shown that the location of the tumor (p < 0.05), histological grade (p < 0.001) and the extent of tumor resection (p < 0.001) have a significant and definitive relationship to the length of survival of pediatric patients with cerebellar astrocytomas. The patient's age (p > 0.05) does not influence the outcome. We could not evaluate the effect of brain stem involvement and adjuvant radiotherapy in our study because of the small sample size.     

Residual or recurrent cerebellar low-grade glioma in children after tumor resection: is re-treatment needed? A single center experience from 1983 to 2003

PURPOSE: The aim of this study was to report on children with cerebellar low-grade glioma (LGG), who were found to have progressive or nonprogresssive residual tumors or tumor recurrence after tumor resection. PATIENTS AND METHODS: Medical records and magnetic resonance imaging (MRI) studies of children (<16 years) with cerebellar LGG were retrospectively analyzed. RESULTS: Of 289 patients with CNS tumors referred between 1983 and 2003, 28 (9.7%) (15 male, 13 female; median age at diagnosis: 71 months) had cerebellar LGG (pilocytic astrocytoma grade I: n = 21; fibrillary astrocytoma grade II: n = 5; mixed hamartoma/pilocytic astrocytoma: n = 1; radiographic diagnosis: n = 1). Total resection was initially performed in 16 patients (57.1%), near total resection in 4 (14.3%), and partial resection in 6 patients (21.4%). One patient underwent biopsy. At a median follow-up of 112 months, 25 patients (89.3%) were alive, 18 of them being in complete remission. Three patients died, 2 due to symptoms related to brain stem compression/infiltration and 1 patient due to postoperative cerebral edema. Presently 5 patients have nonprogressive residual tumors and 2 patients developed nonprogressive recurrences 10 years and 20 months after initial total resection, respectively. None of them required second surgery and none received additional nonsurgical therapies. Only 1 additional patient had to undergo second surgery due to disease progression. CONCLUSIONS: A 'wait and see' strategy is justified in patients with nonprogressive recurrent or residual cerebellar LGG after primary tumor resection. However, long-term follow-up with repeated MRI is mandatory in these patients to detect disease progression. Second surgery is indicated only in patients with unequivocal disease progression, as documented by MRI.     

婴幼儿松果体区肿瘤临床特点及治疗策略

目的探讨婴幼儿松果体区肿瘤患者的临床特点、手术的可行性及手术入路的选择。方法回顾性分析2014年10月至2018年10月期间在广州市妇女儿童医疗中心神经外科接受手术治疗并经病理证实的11例婴幼儿松果体区肿瘤患者的临床资料,包括临床特点、影像学、手术入路、术后病理、并发症等。其中男6例,女5例;年龄3~36个月,平均年龄17.4个月;术前影像学提示均合并梗阻性脑积水。所有患儿均接受手术治疗,其中7例采用经纵裂胼胝体穹隆间入路,1例采用经右侧额中回皮层造瘘入路,1例采用枕下小脑幕上入路,1例采用颞顶皮层造瘘侧脑室三角区入路,1例采用枕下后正中入路。结果11例患儿中,8例患儿达到显微镜下全切除,2例大部分切除,1例仅行活检术。术后病理提示非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumor,AT/RT)3例,松果体母细胞瘤2例,松果体细胞瘤2例,毛细胞星形细胞瘤1例,未成熟畸胎瘤1例,松果体原基瘤1例,混合性胚胎性肿瘤1例。术后脑积水缓解不理想需要行脑室腹腔分流术(ventriculoperitoneal shunt,VP shunt)5例,术后出现硬膜下积液8例,其中行硬脑膜下外引流术好转2例,6例自行吸收,无死亡病例。结论婴幼儿松果体区肿瘤临床病理特点明显与青少年及成人不同,治疗策略制定宜个体化,经纵裂胼胝体穹隆间入路适合大多数婴幼儿患者,明确病理诊断有利于制定下一步的治疗方案。     

Concurrent acute lymphoblastic leukemia and juvenile pilocytic astrocytoma in a pediatric patient

The concurrence of acute lymphoblastic leukemia (ALL) and an asymptomatic juvenile pilocytic astrocytoma is described. A 6-year-old boy without clinical evidence of neurofibromatosis had a juvenile pilocytic astrocytoma diagnosed on radiologic examination and before treatment of acute pre-B cell lymphoblastic leukemia. The patient has had a partial resection of the astrocytoma and is 9 months into treatment of his ALL, which is in complete remission. p53 gene mutation was not identified in this patient. The concurrent diagnosis before treatment of ALL and juvenile pilocytic astrocytoma, the latter normally an indolent tumor, suggests that some cases of astrocytoma previously ascribed to radiotherapy or other treatment may in fact be caused by other factors.     

Second surgery for recurrent pilocytic astrocytoma in children.

Pilocytic astrocytoma (PA) is the most common childhood brain tumor. In cases where the tumor progresses or recurs following primary surgical resection, the appropriate treatment is unclear. Options include chemotherapy, radiation therapy, surgical resection or a combination thereof. To analyze the utility of further surgery, we performed a retrospective, single-institution review of pediatric patients with recurrent PAs from 1990 to 1999 who were treated with a second surgical resection. Patients were excluded if they received adjuvant chemotherapy or radiation therapy. Twenty cases were identified. Tumor locations included: cerebral hemisphere (3), cerebellum (7), optic pathway/hypothalamus (5), thalamus (1) and brainstem (4). The indication for 4 surgeries included an enlarging tumor-associated cyst. At second surgery, 10 of 20 patients had a gross total resection (GTR), 2 a near total resection (NTR), and the remaining 8 patients had a subtotal resection (STR). No patients have died. Two of 10 tumors after GTR, 0 of 2 tumors after NTR, and 7 of 8 tumors after STR had second recurrence/progression at a mean of 15 months (range 4-33 months) following second surgery. The remaining 11 patients are recurrence/progression-free at a mean of 40.7 months (range 19-119 months). Surgery for tumors or midline structures rarely resulted in a GTR (1 of 10 cases). Surgery for tumors located in the cerebral hemispheres or cerebellum resulted in GTR or NTR in all cases and can result in long periods of progression-free survival without further adjuvant treatment.     

Pilomyxoid astrocytoma: neuroimaging with clinicopathologic correlates in 4 cases followed over time

Pilomyxoid astrocytoma (PmA) is a recently identified tumor type characteristically located in the hypothalamus and occurring in young children (<2 y). PmAs were formerly included in large series of pilocytic astrocytomas (PA) until it was realized in 1999 that this differing phenotype tended to manifest a more aggressive biologic course. PmA is defined by its pathologic features of a monomorphous architectural pattern, abundant myxoid background, and absence of features seen in classic PA. We present 4 histologically definite cases of PmA seen at our institution over a minimum 5-year follow-up time: one was rapidly fatal after initial treatment and 2 recurred during therapy. No singular neuroimaging feature can reliably diagnose PmA. However, PmAs tend to be solid, are more commonly necrotic and show extension of abnormal signal intensity into adjacent structures. Cysts, calcification, and perilesional edema are more common in classic PA. Serial neuroimaging in PmA shows early progression of predominantly solid, and later progression of predominantly cystic component. Radiologists should consider this diagnosis particularly in young children with hypothalamic tumors that lack the typical cystic appearance seen in PA.