神经节胶质细胞瘤

神经节胶质细胞瘤孙振荣，赵继宗我院从１９８１年到１９９３年共收治神经系统肿瘤９８００例，其中神经节胶质细胞瘤３１例，发生率为０．３２％。现就这组资料并结合有关文献进行小结。临床资料１．一般情况：本组男１８例，女１３例。年龄分布３～５５岁，平均２３岁。...     

脑干神经节胶质细胞瘤

目的：探讨脑干神经节胶质细胞瘤的临床特点和手术治疗。方法：在我们手术治疗并经病理证实的560例脑干占位病变中，有4例神经节胶质细胞瘤，复习献中报道的24例进行临床分析。结果：脑干神经节胶质细胞瘤的主要发生在儿童及青年人，多位于延髓背部。其生长极为缓慢，病史长，顺MRI上显示为限局性肿瘤影像，显强化，可有囊变及钙化。4例中，1例肿瘤大部分切除，其余3例均完全切除。本组无死亡。结论：在不增加功能障碍的情况下，可将肿瘤完全切除；否则，行部分切除，必要时加行脑室分流手术，也能显延长患生存期。该病不需常规放疗。     

额叶神经节胶质细胞瘤1例

正1病例资料患者,男,48岁,因头痛5 d入院。入院时体格检查:神志清楚,GCS评分15分;两侧瞳孔等大、等圆,直径3 mm,对光反射灵敏;四肢肌力Ⅴ级,肌张力正常,两侧巴氏征阴性。颅脑MRI检查示:右侧额叶囊样信号影,呈T1WI低信号、T2WI高信号、Flair稍低信号,病灶大小约5.2 cm×4.8 cm×4.4 cm,边界清楚,病变外上缘可见小片T1WI稍低、T2WI及Falri高信号     

脊髓神经节胶质细胞瘤三例并文献复习

目的 探讨脊髓神经节胶质细胞瘤的临床特点和手术治疗.方法 收集北京天坛医院神经外科自2010年10月至2011年3月的3例脊髓神经节胶质细胞瘤手术患者资料,结合文献复习进行临床分析.结果 3例患者均以肢体感觉或运动功能障碍起病;MRI检查具低T1高T2信号特征,强化多样,瘤周水肿明显,需与星形细胞瘤、室管膜瘤等其他类型神经上皮来源肿瘤相鉴别;患者行肿瘤全切除或近全切除术,术中肿瘤边界分辨困难;手术病理诊断为神经节胶质细胞瘤、WHOⅠ级,病理切片上混合表达肿瘤性神经节细胞及神经胶质细胞;术后临床症状缓解明显,未行放疗等其他辅助治疗.结论 脊髓神经节胶质细胞瘤相对少见,致残率高,术前诊断困难,手术切除难度大;如手术切除满意预后良好,不需常规行放疔等辅助治疗.     

伽玛刀治疗小脑发育不良性神经节细胞瘤2例报告

目的探讨小脑发育不良性神经节细胞瘤（又称为Lhermitte-Duclos dise,LLD）的临床影像学表现、诊断、伽玛刀治疗。方法分析伽玛刀治疗LLD的临床资料及随访复查资料。结果 LLD的MRI影像呈：沿小脑沟回走行的T1低信号、T2高信号和等信号交替的条纹状分层结构,病灶无强化,病理可确诊,首选手术治疗,伽玛刀治疗后病灶体积缩小,条纹状分层结构影变淡紊乱,能有效控制病情。结论 LLD临床极少见,缺乏临床特征,T2加权像可以诊断,伽玛刀治疗有效。     

促纤维增生性婴儿星形细胞瘤／神经节胶质细胞瘤

促纤维增生性婴儿星形细胞瘤／神经节胶质细胞瘤（DIA／DIG）为临床罕见的低级别神经上皮来源肿瘤（WHOI级）,好发于幕上 ,大多于2岁前发病。     

恶性胶质细胞瘤术后长期存活5例报告

我院1970年～1986年共收治小脑幕上恶性胶质细胞瘤373例,均经病理证实。随访到1987年6月底,发现长期存活5例。报告如下: 例1 刘××女 38岁头痛一年,间断呕吐一月。1970年3月9日入院。检查:双侧视乳头水肿,双眼外展受限,四肢腱反射活跃,无病理征。腰椎穿刺压力2.94kPa,脑脊液蛋白0.29g/L。脑室气造影诊断三脑室肿瘤。1970年4月7日全麻下手术,右额角入路,发现三脑室蛋黄大紫红色肿瘤,囊中切除。病理诊断:室管膜母细胞瘤。术后放疗,全脑2700rad,三脑室4000rad,脊髓300rad。手术后半年恢复机关工作。至今健康情况良好。1987年1月23日脑扫描无复发征象。     

中枢神经细胞瘤94例临床分析

目的 总结中枢神经细胞瘤临床特点,探讨其治疗策略.方法 回顾性分析94例中枢神经细胞瘤患者临床资料.全部行手术治疗,肿瘤全切除66例,近全切除26例,部分切除2例;分析总结中枢神经细胞瘤的临床、病理、影像特点.结果 死亡2例,随访84例,术后放疗56例,肿瘤复发4例.多数患者生存良好.结论 中枢神经细胞瘤多发于室间孔附近侧脑室系统,手术全切除是最佳治疗手段,未全切患者术后放疗可减少复发率.

Abstract：

Objective To analyze the clinical characteristics of central neurocytomas,and discuss the therapeutic strategies.Methods 94 cases of central neurocytomas were studied retrospectively.All patients underwent operation with removal of the tumor through either transcallosal or transcortical approach.Total resection was achieved in 66 patients,subtotal resection in 26 patients and partial resection in 2 patients.The clinical,radiological,histologic and immunohistochemical features of these patients were reviewed and analyzed.Methods Amongthe 94 cases of central neurocytomas,two died after surgery.Among the 84 followed- up cases,56 cases underwent postoperative radiotherapy,4 cases had recurrence.Most patients have favorable prognosis.Conclusion Central neurocytomas occur mostly in the lateral ventricle near the Monro's foramen.Total resection is the best treatment.Postoperative radiotherapy for partially removed tumors may reduce the possibility of recurrence.     

复发性小脑发育不良性神经节细胞瘤一例并文献复习

小脑发育不良性神经节细胞瘤(lhermitte duclos disease,LDD)虽有其特殊的组织病理学改变,但并无特征性临床表现,容易误诊.现报告苏州大学附属第三医院神经外科于2010年10月14日收治的一例复发性LDD患者,回顾性分析其临床症状、影像学检查及治疗过程,结合相关文献进行复习,以提高临床医师对该病的认识.     

颅内神经节胶质细胞瘤1例

患者女性,6岁,2011年11月16日因"反复流涎、右手摸索伴短暂意识障碍20 d"入院.患者入院20 d前无明显诱因出现右手摸索、流涎伴双眼无神,对外界无反应,症状持续约1min后恢复正常,病程中偶感头痛不适,每隔1～2d发作1次,共发作8次,发作间期无其他特殊伴随症状.入院查体:神志清楚,查体合作,语利,双瞳孔等大等圆,直径2.5 mm,对光反射灵敏,伸舌居中,颈无抵抗,两肺呼吸音清,未闻明显及干湿性哕音,律齐,未闻及病理性杂音,腹平软,肝脾肋下未及,移动性浊音阴性,肠鸣音正常,四肢自主活动,病理反射未引出.     

小脑自发性出血的外科治疗(附38例临床分析)

目的探讨小脑自发性出血的显微镜下血肿清除手术方法、治疗效果、预后从而在指导临床治疗及提示预后等方面提供依据方法。方法对38例自发性小脑出血患者手术治疗结果进行回顾性分析。结果 8例小脑自发性出血患者,行枕下后开颅小脑血肿清除术,术后恢复良好33例,重残3例,植物生存1例,死亡1例。结论对自发性小脑出血患者,要根据病情轻重、头颅CT表现等各方面,及时采取手术治疗可以取得良好的治疗效果。     

中枢神经系统神经节细胞胶质瘤组织学及免疫组织化学研究

目的：研究中枢神经系统神经节细胞胶质瘤的组织成分、组织发生、生物学特性及与临床的关系。方法：对２８例神经节细胞胶质瘤进行组织学及免疫组织化学观察。结果：神经细胞大小不等、形态多样、核大、核仁明显，９例可见双核；胶质细胞多为星形细胞，６例含有少枝胶质细胞成分，９例胶质细胞分化不良；血管增生扩张、血管周围淋巴细胞浸润、出血、坏死、囊性变常见。突触素２７例阳性，嗜酪粒蛋白Ａ２３例阳性，神经丝蛋白２５例阳性，神经特异性烯醇化酶、Ｓ－１００、胶质纤维酸性蛋白全部阳性。结论：肿瘤分化与年龄、病程无关；缺乏双核神经元不影响诊断；该肿瘤可能具有神经内分泌功能；本文对该肿瘤的组织发生进行了探讨。     

BRAF V600E mutation is a significant prognosticator of the tumour regrowth rate in brainstem gangliogliomas

BRAF V600E mutations are progression factors in paediatric low-grade gliomas. Furthermore, a high percentage of paediatric brainstem gangliogliomas have BRAF V600E mutations. However, their clinical significance, including possible connections between the biomarkers and ganglioglioma’s clinical features, especially a brainstem counterpart, is unclear. To identify potential molecular features predictive of brainstem ganglioglioma’s clinical outcomes, a retrospective cohort of 28 World Health Organization (WHO) grade I brainstem gangliogliomas was analysed for BRAF V600E, IDH1 R132H, and IDH2 R172K mutations, TERT C228T/C250T promoter mutation, H3F3A K27M mutation and MGMT methylation. The volume of tumours was calculated accurately by using 3D Slicer software. The clinical data of these patients were retrospectively analysed. In tumours with BRAF V600E mutations, the tumour regrowth rate was significantly faster than that of the wild type group (p = 0.001). Moreover, the BRAF V600E mutant group had shorter progression-free survival (PFS) compared with wild type (p = 0.012). On multivariate analysis, no factor was found to be an independent prognostic factor; however, tumours with faster regrowth rates had a strong trend towards an increased risk for shorter PFS (HR = 1.027, p = 0.056). No statistical analysis could be performed to evaluate factors affecting overall survival (OS). These data suggest that BRAF V600E can predict the regrowth rate of brainstem gangliogliomas after microsurgery, and a BRAF V600E-targeted therapeutic may be a promising early intervention measure for patients who harbour BRAF V600E mutation after microsurgery.     

Histopathological and immunohistochemical profile in anaplastic gangliogliomas

BackgroundThe anaplastic ganglioglioma (AG) is the high-grade counterpart of ganglioglioma, a rare mixed tumor composed of neuronal/ganglion and glial cells.Materials and methodsWe describe the histopathology and immunohistochemistry in 7 cases of AG and correlate them with the clinical and radiological features.ResultsOur AG patients correspond to 2.5% of the central nervous system tumor patients evaluated in our institution. The mean age at presentation was 25.7 years, with a male predominance. The most common clinical presentation was generalized tonic–clonic seizures (3/7 cases), in correlation with frequent cortical/subcortical location (6/7 cases). Histopathologically, all our cases showed high-grade features in glial (glial fibrillary acid protein-positive) and neuron-ganglion cells (synaptophysin, PGP-9.5, neurofilament, NSE and CD56-positive), as well as moderate cellularity, frequent mitotic figures and a Ki-67 labeling index >5%. All our patients had poor survival.ConclusionWe found that a typical histopathological and immunohistochemical profile is constant and can be useful in early diagnosis of these aggressive neoplasms.     

Overexpression of the human major vault protein in gangliogliomas

PURPOSE: Recent evidence has been obtained that the major vault protein (MVP) may play a role in multidrug resistance (MDR). We investigated the expression and cellular localization of MVP in gangliogliomas (GGs), which are increasingly recognized causes of chronic pharmacoresistant epilepsy. METHODS: Surgical tumor specimens (n = 30), as well as peritumoral and control brain tissues, were examined for the cellular distribution pattern of MVP with immunocytochemistry. Western blot analysis showed a consistent increase in MVP expression in GGs compared with that in control cortex. RESULTS: In normal brain, MVP expression was below detection in glial and neuronal cells, and only low immunoreactivity (IR) levels were detected in blood vessels. MVP expression was observed in the neuronal component of 30 of 30 GGs and in a population of tumor glial cells. In the majority of the tumors, strong MVP IR was found in lesional vessels. Perilesional regions did not show increased staining in vessels or in neuronal and glial cells compared with normal cortex. However, expression of MVP was detected in the hippocampus in cases with dual pathology. CONCLUSIONS: The increased expression of MVP in GGs is another example of an MDR-related protein that is upregulated in patients with refractory epilepsy. Further research is necessary to investigate whether it could play role in the mechanisms underlying drug resistance in chronic human epilepsy.     

破裂小脑前下动脉动脉瘤的外科治疗

研究背景小脑前下动脉动脉瘤临床罕见,破裂出血后果严重。本文回顾分析12例小脑前下动脉动脉瘤患者(共13个动脉瘤)的临床诊断与治疗经过,分析总结其临床特点、影像学表现、手术方法及预后,以为临床提供参考。方法回顾分析2004年6月-2012年6月治疗的小脑前下动脉动脉瘤患者的临床资料。结果 12例共13个动脉瘤,约占同期颅内动脉瘤总病例数的0.19%(12/6467),平均年龄54岁。动脉瘤形态以囊状居多(10个),梭形少见(3个);分别位于小脑前下动脉起始部即小脑前下动脉基底动脉交界处(3个)、内听道前段(3个,均位于小脑前下动脉第一分叉部)、内听道段(3个)和内听道后段(4个);动脉瘤直径平均为3.90mm。其中3例(4个动脉瘤)行外科手术治疗[动脉瘤夹闭术(2例)、孤立术(1例)],其余9例均行血管内治疗(动脉瘤栓塞术2例、支架辅助动脉瘤栓塞术3例、动脉瘤和载瘤动脉闭塞术4例)。术后并发症包括面瘫(1例)、吞咽困难伴饮水呛咳(1例)和双眼对侧视野缺损(1例);平均随访36.41个月,Glasgow预后分级3分1例、4分2例、5分9例,均达良好标准。结论小脑前下动脉动脉瘤临床罕见,外科手术治疗者需综合评价手术夹闭与血管内治疗适应证,从而选择最佳治疗方法。     

Temporal lobe gangliogliomas associated with chronic epilepsy: Long-term surgical outcomes

Objective

To review the clinical features and surgical outcome in patients with temporal lobe gangliogliomas associated with intractable chronic epilepsy.

Methods

The Rush University Surgical Epilepsy Database was queried to identify patients with chronic intractable epilepsy who underwent resection of temporal lobe gangliogliomas at Rush University Medical Center. Medical records were reviewed for age of seizure onset, delay to referral for surgery, seizure frequency and characteristics, pre-operative MRI results, extent of resection, pathological diagnosis, complications, length of follow-up, and seizure improvement.

Results

Fifteen patients were identified. Average duration between seizure onset and surgery was 14.3 years. Complex partial seizures were the most common presenting symptom. Detailed operative data was available for 11 patients – of these, 90.9% underwent complete resection of the amygdala and either partial or complete resection of the hippocampus, in addition to lesionectomy. Average follow-up was 10.4 years (range 1.6–27.5 years), with 14 patients improving to Engel's class I and one patient to Engel's class III. There were no recurrences, and permanent complications were noted in one patient.

Conclusions

Long-term follow-up of patients with temporal lobe gangliogliomas associated with chronic intractable epilepsy demonstrates excellent results in seizure improvement with surgery and increasingly low incidence of complications with improvements in microsurgical techniques.     

自发性脑出血的临床特征与预后探讨

目的 :对 5 1例自发性小脑出血患者进行临床分析 ,从而进一步探讨其临床特点及预后。方法 :总结 5 1例自发性小脑出血的临床特点 ,结合文献复习对其临床表现、血肿部位、大小、波及范围、血压及预后间的关系进行分析。结果 :首发症状为眩晕呕吐 ,主要病因为高血压病 ,蚓部血肿及入院时收缩压大于 2 0 0mmHg、血肿大于3cm者预后差 ,平均血压与预后无相关性。结论 :小脑出血预后与血肿部位、大小、波及范围及血压密切相关。有规律服药可减轻小脑出血的严重程度。     

髓母细胞瘤的显微手术治疗(附28例报告)

目的 探讨髓母细胞瘤显微手术治疗技巧及其临床疗效.方法 回顾性分析2001年7月至2011年7月显微手术治疗的28例髓母细胞瘤患者临床资料.所有患者术前均行CT和MRI检查.19例经小脑蚓部入路手术,9例经小脑延髓裂入路手术.结果 肿瘤全切除21例(75.0%),次全切除7例(25.0%);出院时GOS评分5分18例(64.3%),4分6例(21.4%),3分3例(10.7%),1分1例(3.6%).结论 髓母细胞瘤以显微手术治疗为主;选择合适的手术入路,术中精细操作避免重要神经结构和血管损伤,可减少术后并发症和提高其临床预后.