视交叉-下丘脑胶质瘤的临床分型及手术治疗

目的　提高视交叉 下丘脑胶质瘤显微手术治疗水平。方法　回顾性分析 16例视交叉 下丘脑胶质瘤的影像学、手术资料及疗效。结果　本组全切除 6例 ,次全切除 4例 ,大部分切除 4例 ;根据肿瘤累及部位 ,视交叉 下丘脑胶质瘤可以分为 5型 :单纯视交叉型、视交叉 视神经型、视交叉 垂体柄型、视交叉 下丘脑型和弥散型 ,各型肿瘤在临床表现及手术学特点方面有所不同。 14例经过 2个月～ 5年的随访 ,除 1例再次手术后死亡其余均存活。结论　根据肿瘤累及部位 ,采用合适的手术技巧积极行显微手术切除 ,术后辅以放射治疗可以使大多数视交叉 下丘脑胶质瘤患者获得长期存活。     

颅内视神经-视交叉胶质瘤的诊断及手术治疗

目的讨论颅内视神经-视交叉胶质瘤的临床特点及手术治疗。方法对我科近6年来手术治疗和病理证实的11例视神经-视交叉胶质瘤患者的临床资料进行回顾性分析。结果视力下降、视野缺损及视神经萎缩是该类肿瘤的主要表现,82%为低级别星形细胞瘤,M RI可良好显示肿瘤生长特点及与周围结构的关系。手术全切除4例、次全切5例、部分切除2例,仅1例术后视力恶化,无死亡病例,总体恢复良好。结论颅内视神经-视交叉胶质瘤有其特点,显微神经外科手术治疗效果好、应该作为该类肿瘤的首选治疗方法。     

视交叉-下丘脑胶质瘤的临床诊治分析

目的探讨视交叉-下丘脑胶质瘤的临床特点及其诊治。方法回顾分析2001年6月至2007年6月我科收治的18例视交叉-下丘脑胶质瘤的临床表现、影像学特征及手术治疗情况。结果18例视交叉-下丘脑胶质瘤患者中，2～23岁16例，32岁和49岁各1例；2例患者伴发神经纤维瘤病Ⅰ型。常见首发症状为头痛。肿瘤次全切除1例，大部分切除13例，部分切除2例，仅活检2例。星形细胞瘤Ⅰ级16例，Ⅱ级2例。术后均辅以放疗和，或化疗。12例随访1～5年，存活9例，死亡3例。结论视交叉一下丘脑胶质瘤需与颅咽管瘤鉴别。手术以改善症状、明确诊断为目的。采取手术、放疗及化疗的综合治疗，患者预后较佳。     

小儿与成人视通路胶质瘤的临床特点

目的探讨小儿与成人视通路胶质瘤(OPG)的临床特点。方法回顾性分析我科2003～2011年收治的24例OPG患者的临床资料。结果本组中小儿患者13例,成人11例;均有视功能损害,伴内分泌症状者分别为4例和7例。按肿瘤部位分型,OPG在小儿和成人中视神经型分别为2例和0例,视交叉型分别为9例和5例,弥散型分别为2例和6例。小儿和成人患者手术治疗分别为12例和11例;术后辅以放化疗分别为2例和6例。小儿均为低级别胶质瘤,成人中3例为高级别或浸润性胶质瘤。术后小儿OPG复发2例,成人5例;小儿患者死亡1例,成人3例。结论小儿OPG好发于视神经及视交叉,以视功能损害为主,性质偏良性,手术效果好,预后较好;成人OPG好发于视交叉及颅内弥散生长,视功能损害和内分泌症状为主,性质偏恶性,单纯手术效果较差,需辅以放、化疗,预后较差。     

105例脑胶质瘤手术治疗体会

目的 探讨脑胶质瘤手术治疗的技术特点及方法。方法总结显微手术治疗105例脑胶质瘤的经验。结果术中全切82例，占77．1％；大部分切除23例，占22．9％；术中无一例死亡。结论对脑胶质瘤要认识到手术全切的重要性，术前综合分析，术中遵循一看、二摸、三穿刺、四快速活检的原则，是提高手术疗效，减少并发症及复发的良好保证。     

视神经损伤的显微手术治疗

目的总结视神经损伤的显微手术治疗经验。方法对13例颅脑损伤致视力障碍病人行经翼点入路手术．打开硬膜后在显微镜下清除骨折碎片，磨开骨性视神经管，开放视神经鞘，切开镰状韧带，清除视交叉周围的血肿。结果术后随访2-6个月．9例有效，总有效率69．2％。结论经颅显微手术治疗颅脑损伤所致视神经损伤可以取得良好的效果；视神经管有无骨折不是决定手术的主要依据。     

丘脑胶质瘤的临床特征与手术治疗的分析

目的研究丘脑胶质瘤的临床特征、诊疗方法和手术技巧。方法回顾性分析我院经手术治疗的39例丘脑胶质瘤患者的临床表现、肿瘤影像学特点和手术治疗效果。结果丘脑不同类型胶质瘤影像学表现、全切率不尽相同。病程7d～3．5年，平均3．1月。肿瘤颅内压增高症状最为常见（90％），轻偏瘫56％，不自主运动10％，智力减退或精神症状30％；全组患者肿瘤手术全切率为53％，次全切率为29％，部分切除率为18％；出院时好转、稳定者占79％，恶化及出现新体征者占21％；手术死亡率为0。结论MRI是目前诊断丘脑肿瘤的最好检查方法。肿瘤的病理分类与临床症状、影像特征及手术效果均有关。     

经终板入路显微切除视交叉后肿瘤

目的 探讨视交叉后肿瘤的手术入路和显微手术技巧。方法 采用终板入路显微切除11例视交叉后肿瘤，其中颅咽管瘤7例，垂体腺瘤、血管瘤、下丘脑胶质瘤和视交叉胶质瘤各1例。结果 全切除10例、次全切除1例、良好9例、死亡2例。随访2个月至2年未见肿瘤复发。结论 应用娴熟的显微手术技巧，经终板入路彻底切除视交叉后肿瘤对下丘脑无明显损伤，可以取得良好的临床疗效。     

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。     

脊髓髓内肿瘤显微手术疗效的长期随访

目的：总结脊髓髓内肿瘤的手术经验，对手术治疗脊髓髓内肿瘤的价值及疗效进行评价。方法：对两个神经外科中心手术治疗的123例脊髓髓内肿瘤进行回顾性分析，对肿瘤部位、病理类型、手术切除程度、手术 前后临床症状与体征的变化，进行长期随访，以评估手术疗效。结果：本组室管膜瘤占脊髓髓内肿瘤的34．1％，手术全切除率为90．5％，次全切除率为7．1％，部分切除及活检率为2．4％；星形细胞瘤占26．8％，全切除率为30．3％，次全切除率为39．4％，部分切除及活检率为30．3％；脂肪瘤占14．6％，全切除率为11．1％，次全切除率为38．9％，部分切除或活检率为50．0％；血管网状细胞瘤占10．6％，全切除率为76．9％，次全切除率为23．1％；其它肿瘤占13．9％，全切除率为82．4％，次全切除率为11．7％，部分切除及活检占5．9％。94例经平均38．6个月长期随访，89例存活，5例死亡。结论：脊髓髓内肿瘤以良性及低恶性度肿瘤多见，早期手术预后较好，应力争在脊髓功能未出现严重损害前手术，以争取得到较好的手术疗效。     

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.     

儿童颅内胶质瘤临床特点回顾性分析

目的探讨儿童颅内胶质瘤的临床特点、治疗方法及预后。方法对2000~2013年收治的95例14颅内胶质瘤患儿的临床资料进行回顾性分析。结果肿瘤位于幕上50例、幕下45例。保守治疗20例,显微手术治疗55例,伽马刀治疗20例。55例手术患者中,肿瘤全切42例(76.4%),次全切13例(23.6%);术后病理学结果示胶质瘤WHO(2007)Ⅰ~Ⅱ级34例(61.8%),Ⅲ~Ⅳ级21例(38.2%)。48例患儿随访3~108个月,平均时间37.8月;保守治疗的2例患儿2年内均死亡;6例肿瘤全切患儿于术后3~15个月复发;其余40例随访期间未复发。结论儿童胶质瘤好发于近中线处,病理学类型以星形细胞瘤及髓母细胞瘤为主,治疗应以手术全切肿瘤为主,适当辅以化疗,预后相对较好。     

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.     

The moyamoya syndrome associated with irradiation of an optic glioma in children: Report of two cases and review of the literature

We report two cases of the moyamoya syndrome which became clinically apparent after irradiation of an optic glioma during childhood. A summary of 14 cases of this syndrome following irradiation of intracranial tumors is also presented. Nine of these cases were optic gliomas; five were found in children with neurofibromatosis, another disorder that has a strong association with the moyamoya syndrome. The effectiveness of irradiation of optic gliomas in childhood is not definitely established. The possibility of inducing serious vascular disease is a further reason for caution when considering irradiating these tumors.     

小儿下丘脑胶质瘤的诊断和治疗

目的为探讨小儿下丘脑胶质瘤的临床特点、诊断及治疗。方法对1996年11月至2001年9月间收治的27例小儿下丘脑胶质瘤作回顾性分析。结果27例小儿下丘脑胶质瘤占同期小儿颅内肿瘤的1．9％，占鞍区肿瘤的6．9％。临床表现主要为颅内压增高和下丘脑损害。27例中全切除4例和次全切除17例和大部分切除6例，无死亡病例。其中21例术后行放疗。随访23例，效果满意19例，生活能自理3例，植物生存1例。结论小儿下丘脑胶质瘤诊断应根据临床表现及神经放射学检查综合分析。显微外科手术治疗是主要有效的治疗方法，未全切除术后应放疗。     

BRAF Duplications and MAPK Pathway Activation Are Frequent in Gliomas of the Optic Nerve Proper

ABSTRACT: Optic pathway gliomas represent a specific subtype of astrocytoma with unique clinicopathologic and biologic properties, but studies of tumors in the optic nerve proper have been hampered by limited tissue availability. We analyzed optic nerve gliomas of 59 patients (median age, 9 years; range, 3 months-66 years; 33 female, 26 male) using formalin-fixed paraffin-embedded material in tissue microarrays. Seven patients had the clinical diagnosis of neurofibromatosis type 1 (NF1). Fluorescence in situ hybridization studies were performed for BRAF, PTEN, CDKN2A (p16), and NF1. Immunohistochemistry was performed for glial fibrillary acidic protein, phospho-ERK, and mutant IDH1 protein. The BRAF duplication was present in 11 (73%) of 15 evaluable tumors, including 1 NF1 patient (1 of 4 tested; 25%). The single tumor lacking BRAF duplication or NF1 association had histologic features of a ganglioglioma. Conversely, heterozygous PTEN deletions were present in 2 (8%) of 25 evaluable cases, one of which was BRAF duplicated and the other was NF1 associated. CDKN2A and NF1 deletions were absent in all tumors tested. Phospho-ERK immunoreactivity was present in 55 (96%) of 57 tumors and was mostly strong and diffuse (80%). Only 1 case of 53 expressed IDH1. Thus, optic nerve gliomas demonstrated molecular alterations typical of pilocytic astrocytomas, including the universal presence of either BRAF duplication or NF1 association and common mitogen-activated protein kinase pathway activation but very rare mutant IDH1 expression.     

The role of gamma knife radiosurgery in children

OBJECTS: Despite advances in microneurosurgery, the surgical management of deep-seated lesions is still associated with a high risk. Gamma knife radiosurgery (GKRS), however, has improved the outcome of cerebral tumors and arteriovenous malformations (AVMs) in delicate areas. METHODS: Between March 1992 and June 1998, 50 children (age 1-16 years) with intracranial lesions were treated with GKRS. There were 12 low-grade gliomas, 12 high-grade gliomas, 7 craniopharyngiomas, 3 hamartomas, 2 meningiomas of the skull base, 2 vestibular schwannomas, 1 pituitary adenoma, 1 choroid plexus papilloma, and 10 AVMs. The mean size of the pathologies was 4.6 cm3 (range: 0.21-25.5 cm3). A mean marginal dose of 16 Gy (8-25 Gy) was applied to a mean isodose surface of 50% (35-90%). Clinical and neuroradiological follow-up were analyzed for outcome. Follow-up periods of 45 of these patients ranged from 8 to 79 months (mean 36 months); 5 patients were lost to follow-up. Neoplasms decreased in size in 15 cases (41%), remained unchanged in 13 patients (35%), and increased in 9 cases (24%). AVMs obliterated in 3 children (38%) within 24 months. Neurological follow-up demonstrated improved clinical status in 7 patients (15.5%), stable neurological status in 31 cases (69%) and clinical deterioration in 7 patients (15.5%). The treatment was well tolerated and no complications occurred. CONCLUSIONS: GKRS represents a safe and effective treatment option for benign neoplasms or AVMs in pediatric patients and may extend survival times of children with malignant lesions.     

Ascites following ventriculoperitoneal shunting in children with chiasmatic-hypothalamic glioma

OBJECT: Optic pathway gliomas in children can involve the optic nerve, chiasm, and hypothalamus. This uncommon, slowly growing tumor can cause hydrocephalus, which usually requires placement of a ventriculoperitoneal (VP) shunt. Symptomatic ascites may occasionally develop as a complication of the VP shunt procedure. The purpose of this study was to assess the risk factors associated with CSF ascites in children with optic pathway gliomas. METHODS: Twenty-two children (ages 4 months to 20 years) with chiasmatic-hypothalamic optic gliomas participated in this study. Four children were diagnosed with a chiasmatic glioma, 7 with a hypothalamic glioma, and 11 with a glioma involving both the optic chiasm and hypothalamus. Twelve children (55%) developed hydrocephalus and required VP shunt placement. Of the 12 shunted children, 4 (33%) developed CSF ascites. The incidence of ascites was not associated with infection, tumor metastasis, or multiple shunt revisions. There was no correlation with the size of the tumor. All 4 children with ascites had tumor involving the optic chiasm or optic nerve. None of the 5 children with pure hypothalamic glioma who underwent VP shunt placement have developed ascites. Among the 7 children suffering from chiasmatic or optic nerve gliomas who developed hydrocephalus, the risk of developing ascites as a complication of VP shunt placement was 57% (4/7). Ventriculoatrial (VA) shunt was the treatment of choice for children with VP shunt-induced ascites. After placement of a VA shunt the ascites subsided. The children did not develop further complications. CONCLUSION: We conclude that glioma involving the optic chiasm or nerve is associated with a high risk of developing ascites following VP shunt placement. VA shunt may be the treatment of choice for children with chiasmatic or optic nerve gliomas who require a CSF diversion procedure.     

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。