视神经胶质瘤

视神经胶质瘤罕见 ,大部分视神经胶质瘤发生于 2 0岁内。临床自然发展病程为 :因肿瘤生长引起视力障碍甚或死亡。本文就其病理学特点、生长部位、临床表现、与神经纤维瘤病的关系、治疗方法及预后作一综述。     

视交叉胶质瘤的诊断和显微外科治疗

目的探讨视交叉胶质瘤的临床表现、诊断要点、病理及治疗方案。方法回顾性分析13例经手术和病理证实的视交叉胶质瘤。结果本组占同期鞍上胶质瘤的2l％;青少年病人占大多数。视力下降、视野改变为主要症状;MRI可良好显示病灶特点及肿瘤与邻近结构的关系。手术采用一侧冠状切口经额下入路和(或)经纵裂入路,囊内切除肿瘤,充分内减压;术后行常规放射治疗。病理证实视交叉胶质瘤多为毛细胞型星形细胞瘤。结论视交叉胶质瘤具有一定的临床特征,显微手术切除疗效好。     

儿童眶内视神经胶质瘤的手术治疗和随访

目的 探求儿童眶内视神经胶质瘤的手术方法.方法 总结3例15岁以下(分别是1.5岁、3岁和10岁)眼眶内视神经胶质瘤临床症状和体征,肿瘤影像学发现,采用经额底入路打开前颅底(眶顶),切除视神经肿瘤.根据肿瘤切除程度和患儿年龄采用术后放疗.结果 3例患儿视力下降,2例有下凸眼,患侧瞳孔扩大且无光反射.2例肿瘤向颅内生长其视乳头呈苍白色,1例眶内局限生长视乳头呈水肿改变.MRI和CT显示3例均为右侧眶内肿瘤,呈梭状外观,其中2例肿瘤向颅内生长,但没有超过视交叉.经冠状切口额底入路,去除眶顶板,眶内段肿瘤全部切除3例,其中2例向颅内生长部分没有全切除.术后1例10岁患儿行放疗,另2例为3岁以下患儿,未行放疗.3例患儿术后连续随访,最长8年,均没有肿瘤复发.结论 对于局限存眶内段的视神经胶质瘤应予全切除,对4岁以上患儿,如有肿瘤颅内生长,应行术后放疗,患儿可获得长期生存.     

视神经胶质瘤的手术治疗(附6例报告)

我院自1989年9月～2002年2月共收治视神经胶质瘤(optic pathway glioma ,OPG)患者6例,现结合文献报告如下。一、对象与方法1.一般资料:本组男4例,女2例。年龄6～20岁,平均10.6岁。6例均有不同程度的视力下降、视野缺损、视神经萎缩,头痛、呕吐5例,眼球运动障碍伴眼震2例,生长发育迟缓3例,多饮、多尿1例。1例合并1型神经纤维瘤病。     

儿童视神经-下丘脑胶质瘤的治疗和预后分析(附7例报告)

儿童视神经 下丘脑胶质瘤发病率低 ,临床罕见 ,我们从 1 993年至 2 0 0 0年共收治病人 7例 ,现报告如下 :临床资料　　 1 .一般资料 :男 5例 ,女 2例。年龄 1 0～ 1 5岁。平均年龄 1 2 5岁 ,临床症状主要表现为视力减退 7例 ,视野缺损或单盲 6例 ,眼底视神经萎缩7例 ,均无内分泌改变及颅内压增高症状。影像学检查 :CT平扫为略低密度或等密度影 ,增强后轻度强化 ,肿瘤边界清楚 ,以纺锤状生长多见。MRI扫描 :T1WI扫描为低或略低信号 ,T2WI为高或混杂信号 ,肿瘤边界清楚 ,往往侵犯视交叉 ,部分沿视束向后生长 ,侵犯下丘脑。肿瘤直径为 3 …     

低级别视神经胶质瘤的诊治和预后

视神经胶质瘤发病率较低，临床上少见，作从1993年至2000年共收治核病患9例，现报告如下。     

替莫唑胺治疗视神经胶质瘤的疗效观察

目的：观察和探讨替莫唑胺治疗视神经胶质瘤的疗效。方法对我院2010-01-2013-01收治的40例视神经胶质瘤患者的临床资料进行回顾性分析,患者均使用外科手术进行治疗,按随机数字表法将患者随机分为观察组和对照组,每组20例,观察组在术后使用替莫唑胺进行化疗,对照组在术后未给予替莫唑胺,对2组患者的疗效进行对比、分析。结果经化疗后,观察组肿瘤病灶完全缓解8例,部分缓解5例,病情稳定4例,有效率为85％;随访中观察组无复发。对照组在随访中有4例出现视神经胶质瘤复发,为多形性母细胞瘤。观察组的视力恢复情况优于对照组,肿瘤复发率低于对照组。经治疗后,观察组出现脑水肿1例,恶心、呕吐1例;对照组脑水肿7例,血液学毒性4例,恶心、呕吐6例,2组对比差异具有统计学意义（ P＜0.05）。结论作为新型抗肿瘤药物,替莫唑胺具有不良反应小,血脑屏障通透性能优良,耐受性佳,耐药率较低等优点,在视神经胶质瘤患者术后化疗效果显著,值得临床推广使用。     

儿童丘脑胶质瘤的显微外科治疗

目的 探讨儿童丘脑胶质瘤的临床特征和显微外科治疗.方法 总结49例儿童丘脑胶质瘤的临床特征,分别采取经胼胝体-穹窿间入路、胼胝体-侧脑室入路、经顶或顶枕-侧脑室(经三角区)入路、经额-侧脑室入路、经颞皮层入路、经颞下小脑幕入路进行显微外科治疗.结果 初次手术中,近全切除23例,大部切除17例,部分切除9例,围手术期死亡2例.39例获得随访,16例存活,其中15例生活可以自理.结论 手术是儿童丘脑胶质瘤的首选治疗,根据肿瘤的位置、生长方向及术者经验采取合理的手术入路,尽可能减少重要结构的损伤,减少术后并发症.     

儿童脊髓髓内胶质瘤的显微外科治疗

目的 总结儿童脊髓髓内胶质瘤显微神经外科治疗的经验.方法 1999-2005年共手术治疗儿童脊髓髓内胶质瘤52例,男30例,女22例,年龄2～14岁(平均7.8岁),病程2个月-3年;肿瘤直径占脊髓横断面的60%～90%,长度跨越4～11个椎体;临床表现按McCormick神经功能分级:Ⅰ级8例,Ⅱ级35例,Ⅲ级7例,Ⅳ级2例.早期采用传统椎板切除术、后期采用整块切除棘突及椎板后再回植重建的方法进入椎管,在显微镜下沿脊髓后正中裂切开脊髓显露肿瘤并分块切除.结果 星形细胞瘤35例(67%),其中全切26例(74%),部分切除9例(26%);室管膜瘤15例(29%),均全部切除;节细胞瘤2例(4%),其中1例全切,1例部分切除.术后近期症状较术前加重或出现新神经损害症状者29例(56%),症状改善或无变化者23例(44%);随访6个月-6年,38例恢复正常生活或学习,10例可自理生活,4例不能自理生活.肿瘤复发2例.患儿功能预后与术前神经功能分级密切相关,术前神经损害症状越轻,术后恢复越好.结论 显微神经外科手术切除脊髓髓内胶质瘤是目前最有效的治疗措施,最佳手术时机是在患儿尚未发生严重神经损害症状之前.     

视神经鞘脑膜瘤(附5例报道)

目的 探讨显微神经外科技术在治疗视神经鞘脑膜瘤中的作用。方法 5例视神经鞘脑膜瘤均采用显做外科切除，1例采用额颞入路，2例为改良翼点入路，2例经眶额-翼点入路。术中力求解剖保留视神经。对比手术前后视力改变，以评价手术效果。结果 肿瘤全切3例，视神经解剖保留4例。术后视力情况：1例较术前进步，1例无变化，3例完全失明。结论 显做神经外科技术多可以解剖保留视神经，但很少能保留视力。     

神经导航辅助显微手术在等体积切除脑中央沟周围胶质瘤中的价值

目的探讨神经导航辅助显微手术在等体积切除脑中央沟周围胶质瘤中的价值。方法应用神经导航系统，术前标定肿瘤边界、术中实时指导，对16例脑中央区胶质瘤患者行等体积显微切除手术。结果术后影像复查显示：肿瘤全切除14例，次全切除2例。术后神经功能缺损改善或无变化13例；神经功能缺损短期加重3例，3个月后恢复到术前水平，无手术死亡。结论在脑中央沟周围胶质瘤的显微手术中，神经导航系统有助于提高肿瘤的影像学全切除率，减轻对脑组织的损伤，改善神经功能缺失。     

纯岛叶低级别胶质瘤的显微外科治疗

目的探讨纯岛叶低级别胶质瘤的显微手术方法及疗效。方法回顾性分析17例纯岛叶低级别胶质瘤病人的临床资料。其中肿瘤位于左侧岛叶8例,右侧岛叶9例,均采用翼点外侧裂入路手术切除肿瘤。结果肿瘤全切除12例,近全切除5例。病理结果:星形细胞瘤8例,少枝胶质细胞瘤4例,少枝星形细胞瘤5例。无手术死亡,术后早期发生偏瘫、失语各2例。随访17例,时间6~12个月,均未见肿瘤复发,轻偏瘫1例,轻度失语1例。结论应用显微外科技术经翼点外侧裂入路治疗纯岛叶低级别胶质瘤安全、有效。了解岛叶解剖有助于最大程度切除岛叶低级别胶质瘤,减少病人术后神经功能障碍。     

Diagnostic pitfall: Optic neuritis mimicking optic nerve glioma

We report a case of optic neuritis with enlargement of the optic nerve on MRI. This neuroradiological finding is most commonly seen in tumours of the optic nerve and led together with an inconclusive intraoperative frozen section to an incorrect tentative diagnosis of optic nerve glioma which fatally resulted in the resection of the optic nerve.     

儿童脑膜瘤(附25例报告)

目的总结儿童脑膜瘤的临床特点和治疗经验。方法回顾分析过去6年间我科收治的25例儿童脑膜瘤的临床资料。占同期儿童颅内肿瘤的1.6%,其中发病年龄以10～15岁居多,平均11.3岁,男女比例接近。结果本组25例肿瘤中,位于脑室系统内和后颅凹者各占20%;肿瘤位于脑皮层下、与硬膜无粘连者占24%;显微手术肿瘤全切除和近全切除为92%。结论儿童脑膜瘤具有与成人不同的特点,其预后与多种因素相关,早期诊断和手术切除可取得良好疗效。     

Optic pathway gliomas in children with and without neurofibromatosis 1

Optic pathway gliomas represent 2 to 5% of brain tumors in children. Frequently asymptomatic, sometimes they demonstrate rapid growth, causing considerable visual dysfunction, neurologic deficits, and endocrine disturbances. Most optic pathway gliomas are diagnosed in patients with neurofibromatosis 1. Little is known about their natural course; therefore, there are no clear and widely accepted guidelines for their treatment. This study compared the clinical manifestations and natural history of sporadic and neurofibromatosis 1-associated optic pathway gliomas with regard to age at diagnosis, gender, and findings on neurologic, ophthalmologic, and neuroradiologic examinations in 83 children with optic pathway gliomas: 51 children with neurofibromatosis 1 and 32 children without any symptoms or signs of neurofibromatosis 1. A prospective study was performed in 21 patients with neurofibromatosis 1. In the rest of the patients with neurofibromatosis 1 and in 32 children with sporadic tumors, the analysis was carried out retrospectively. There was an increased incidence of females in the group of patients with neurofibromatosis 1 with optic pathway gliomas compared with the entire group of patients with neurofibromatosis 1 remaining for follow-up (P = .013). All optic pathway gliomas were found in children below 10 years of age, slightly earlier in the group without neurofibromatosis 1 (median age 4.6 vs 4.8 years). Children with optic pathway gliomas associated with neurofibromatosis 1 had predominantly multifocal lesions (P = .0001), whereas in the group without neurofibromatosis 1, isolated chiasmal involvement was more common (P = .002). Children with sporadic gliomas had significantly more frequently increased intracranial pressure, decreased visual acuity, and abnormalities of fundus of the eye at the time of diagnosis. The radiologic progression, visual deterioration, and endocrinologic complications were documented on follow-up more commonly in children with sporadic tumors. Our findings support the concept that there is an earlier and more severe clinical presentation of optic pathway gliomas in children with sporadic tumors than in those associated with neurofibromatosis 1.     

Brainstem gliomas

Introduction Brainstem gliomas have historically been one of the most difficult pediatric cancers to treat. Tumors arising in the brainstem were once uniformly discounted as surgically unresectable lesions. Early neurosurgeons thought this location to be inoperable and fraught with disaster. The advent of computed tomography (CT), magnetic resonance imaging (MRI), and sophisticated neurophysiological monitoring techniques have significantly advanced the surgical treatment of these precarious lesions.Review Brainstem gliomas are now recognized as a heterogenous group of tumors. They have been broadly classified into several categories depending upon the classification scheme. All these classification systems provide a framework to predict growth patterns, surgical resectability, and overall prognosis of these tumors. These systems allow the surgeon to obtain a better understanding of the distinction between low-grade tumors and diffuse inoperable tumor types. The authors review the current literature and management of brainstem tumors.     

Brain gliomas,hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1

PurposeTo evaluate the incidence and clinical importance of brain gliomas – optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis.Subjects and methodsWe analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery.ResultsWe have found OPGs in 77/285 (27%) children and GOOPs in 29/285 (10,2%) of NF1 children, of who 19 had OPG and GOOP together, so the total number of brain glioma was 87/285 (30,5%). GOOPs were significantly more often treated than OPGs (p > 0.01). OPGs contain clinically important subgroup of 14/285 (4.9%) spreading to hypothalamus. Spontaneous regression was documented in 4/285 (1.4%) gliomas and the same number of NF1 children died due to gliomas.Obstructive hydrocephalus was found in 22/285 (7.7%) patients and 14/22 cases were due to glioma. Idiopathic aqueduct stenosis caused hydrocephalus in 6/22 cases and was found in 2.1% of NF1 children. Two had other cause.ConclusionsThe total brain glioma number (OPGs and only GOOPs together) better reflected the overall brain tumour risk for NF1 children. However, GOOPs occur less frequently than OPGs, they are more clinically relevant. The obstructive hydrocephalus was severe and featuring frequent complication, especially those with GOOP. Idiopathic aqueduct stenosis shows an unpredictable cause of hydrocephalus in comparison with glioma and is another reason for careful neurologic follow up.