vestibular schwannomas

前庭神经鞘瘤
  • 文章类型: Journal Article
    这项研究的目的是初步探讨手术的安全性,功效,技术,和完全神经内镜手术通过乙状窦后入路切除桥小脑角(CPA)肿瘤的临床价值。回顾性分析2014年6月至2023年6月采用全神经内镜手术治疗的47例桥小脑角区(CPA)肿瘤的临床资料。根据手术时间等指标评估手术技术的疗效和优势,神经内镜技术,术中神经和血管的完整性,肿瘤切除范围,结果或术后症状,和并发症的发生率。47例桥小脑角肿瘤包括34例表皮样囊肿,前庭神经鞘瘤7例,脑膜瘤6例。所有患者均接受完全神经内镜手术。使用单刀两手技术切除了20个肿瘤,使用两名外科医生的四手技术切除了27个肿瘤。在所有47例中,受影响的颅神经的解剖完整性均得到保留。没有病人术后出血,脑脊液漏,无菌性或化脓性脑膜炎,或死亡。肿瘤总切除率为72.3%(34/47),症状改善率为89.4%(42/47)。所有患者均随访2~12个月。没有人死亡,也没有任何肿瘤复发的迹象。通过分析我们中心使用乙状窦后入路对CPA肿瘤进行的47例完全神经内镜切除,我们相信这种方法允许完成,安全,有效切除CPA肿瘤,值得临床推广。
    The objective of this study is to preliminarily investigate the surgical safety, efficacy, techniques, and clinical value of fully neuroendoscopic surgery for the resection of cerebellopontine angle (CPA) tumors via a retrosigmoid approach. The clinical data of 47 cerebellopontine angle area (CPA) tumors that were treated by full neuroendoscopic surgery from June 2014 to June 2023 were retrospectively analyzed. The efficacy and advantages of the surgical techniques were evaluated based on indicators such as duration of the surgery, neuroendoscopic techniques, intraoperative integrity of nerves and blood vessels, extent of tumor resection, outcomes or postoperative symptoms, and incidence of complications. The 47 cases of cerebellopontine angle tumors include 34 cases of epidermoid cysts, 7 cases of vestibular schwannomas, and 6 cases of meningiomas. All patients underwent fully neuroendoscopic surgery. Twenty tumors were removed using the one-surgeon two-hands technique, and 27 tumors were removed using the two-surgeons four-hands technique. The anatomical integrity of the affected cranial nerves was preserved in all 47 cases. None of the patients suffered a postoperative hemorrhage, cerebrospinal fluid leak, and aseptic or septic meningitis, or died. The rate of total tumor resection was 72.3% (34/47), and the symptom improvement rate was 89.4% (42/47). All patients were followed up for 2 to 12 months, and none died nor showed any signs of tumor recurrence. By analyzing 47 fully neuroendoscopic resections of CPA tumors using the posterior sigmoid sinus approach in our center, we believe that such method allows complete, safe, and effective resection of CPA tumors and is thereby worthy of clinical promotion.
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  • 文章类型: Journal Article
    目的:探讨经耳道经胸内镜入路(TEA)全内镜下切除丙炔神经鞘瘤(ILS)的方法和疗效。研究设计:回顾性病例回顾。设置:医院。患者:所有受ILS影响的患者,没有延伸到内耳道,并于2020年在我们医院接受了TEA手术。干预:治疗性。主要结果衡量标准:恢复状态,术后并发症及术后残留症状。结果:纳入3例患者,所有这些都进行了总体全面切除。随访时间为10个月至2年。术中及术后均未发生重大并发症。术后无面瘫或脑脊液漏。TEA的住院时间为5天。3例患者在未接受前庭治疗1周后眩晕缓解。只有1例患者抱怨攀爬或握住重物时出现短暂的眩晕发作。结论:TEA具有清晰的视觉来识别解剖结构的优点,能够完全切除肿瘤,减少操作时间,术后恢复更快。证据等级:IV。
    Objective: To investigate the technique and efficacy of fully endoscope resection of intralabyrinthine schwannomas (ILS) by transcanal transpromontorial endoscopic approach (TTEA). Study Design: Retrospective case review. Setting: Hospital. Patients: All patients who were affected by ILS, without extension to the internal auditory canal and underwent surgery with TTEA in our hospital in 2020. Intervention(s): Therapeutic. Main Outcome Measure(s): Recovery status, postoperative complications and remaining symptoms after surgery. Results: Three patients were included, all of which underwent gross total resections. The follow-up period was from 10 months to 2 years. No intraoperative and postoperative major complications were observed. There was no facial paralysis or cerebrospinal fluid leakage postoperatively. The hospitalization time of TTEA was 5 days. Three patients\' vertigo was relieved after 1 week without receiving vestibular therapy. Only 1 patient complained of transient episodes of vertigo when climbing or holding heavy objects. Conclusions: TTEA has the advantages of clear vision to identify the anatomical structure, enabling complete tumor resection, reduced operation time, and faster postoperative recovery.Level of Evidence: IV.
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    文章类型: Journal Article
    背景:前庭神经鞘瘤是桥小脑角区最常见的良性肿瘤。随着肿瘤的生长,它通常会由于附近神经的压迫而导致严重的听力损失,导致生活质量下降。本研究通过文献计量和可视化分析检查了前庭神经鞘瘤相关研究,探索了当前的趋势和研究热点。
    方法:使用WebofScienceCoreCollection检索了2002年至2021年发表的与前庭神经鞘瘤有关的研究。利用R软件对数据进行处理和可视化分析,VOSviewer,和城市空间。
    结果:本研究共纳入3,909篇出版物,出版物年产量总体呈上升趋势。美国,德国,英国是最多产的国家,发表最多的文章德国的国际合作最频繁,中心性得分最高。梅奥诊所,加州大学,和哈佛大学是三个最有生产力的机构。耳科学和中子学是最多产的期刊,MJLink是最有成效和得分最高的中心性作者。当前的前沿主题主要集中在“听力保护”和“放射外科”。主题趋势图和主题图显示“听力损失”,\"眩晕\",“磁共振成像”,\"放射外科\",“立体定向放射外科”,和“伽玛刀”是当前讨论的焦点主题。
    结论:听力保护是该领域当前的前沿课题。放射外科在前庭神经鞘瘤领域具有广阔的前景。立体定向放射外科是全球关注的焦点。文献计量和可视化分析为前庭神经鞘瘤领域提供了独特而客观的视角,并可能有助于学者们确定新的研究方向。
    BACKGROUND: Vestibular schwannoma is the most common benign tumor in the pontocerebellar horn region. As the tumor grows, it often causes severe hearing loss due to compression of nearby nerves, resulting in a lower quality of life. This study examined vestibular schwannoma-related research through a bibliometric and visualization analysis, and it explored current trends and research hot spots.
    METHODS: Research related to vestibular schwannoma published from 2002 to 2021 was searched using the Web of Science Core Collection. The processing and visualization analysis of the data were conducted using R software, VOSviewer, and CiteSpace.
    RESULTS: A total of 3,909 publications were included in this study, and an overall increasing trend in the annual output of publications was found. The United States, Germany, and the United Kingdom were the most prolific countries, publishing the most articles. Germany had the most frequent international cooperation and the highest centrality score. The Mayo Clinic, University of California, and Harvard University were the three most productive institutions. Otology & Neurotology was the most prolific journal, and MJ Link was the most productive and highest scoring author for centrality. Current frontier topics mainly focused on \"hearing preservation\" and \"radiosurgery\". A map of trends in topics and a thematic graph revealed that \"hearing loss\", \"vertigo\", \"magnetic resonance imaging\", \"radiosurgery\", \"stereotactic radiosurgery\", and \"gamma knife\" were the topics of focus of current discussions.
    CONCLUSIONS: Hearing preservation is a current frontier topic in this area. Radiosurgery has a promising future in the field of vestibular schwannoma, and stereotactic radiosurgery is a focus of global attention. Bibliometric and visualization analysis offer a unique and objective perspective of the field of vestibular schwannoma and may assist scholars in the identification of new research directions.
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  • At present, the main treatment for vestibular schwannomas is surgery. Considering the risk of multiple complications from surgery and the subjective and objective conditions of patients, a non-surgical treatment modality, namely stereotactic radiotherapy, has gradually been included in the treatment of vestibular schwannomas. Studies have shown that Gamma Knife therapy has a more prominent therapeutic effect on smaller tumors and can alleviate facial nerve disorders caused by space occupying of tumor mass. Cyberknife not only has a better effect on tumor control, but also has an ideal retention rate for patients\' auditory function. Proton beam therapy has also been gradually applied to the treatment of vestibular schwannomas, but the effect of treatment remains to be further studied. Drug therapy includes a variety of target inhibitors and anti-angiogenic drugs. At present, drug treatment focuses more on preclinical research. This article reviews the clinical research of various radiotherapy and the progress of drug treatment.
    摘要: 目前听神经瘤的治疗方式以手术治疗为主,考虑到手术带来的多种并发症风险和患者的主/客观条件,非手术治疗中的立体定位放射治疗逐渐成为听神经瘤治疗的一部分。研究表明,伽马刀治疗对较小的肿瘤治疗效果较突出,并可缓解因肿瘤占位带来的面神经及听神经疾病。赛博刀不仅对肿瘤的控制效果较好,而且患者的听功能保留率也较为理想。质子束疗法也逐渐应用于听神经瘤的治疗,但还需进一步研究。药物治疗(包括多种靶点抑制剂与抗血管生成药物)目前多聚焦于临床前研究。本文对多种放射治疗临床研究与药物治疗进展进行综述。.
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  • 文章类型: Journal Article
    前庭神经鞘瘤(VS)是中枢神经系统最常见的良性肿瘤之一。目前,VS的流行治疗方法包括手术,立体定向放射治疗,和后续观察,等。然而,仍然没有可用于治疗VS的药物疗法。虽然手术技术相对成熟,并发症无法完全避免。此外,不同病例的生长速度和患者对放疗的敏感性差异很大。随着分子生物学研究的不断进步,关于VS生长机制的研究大多集中在神经纤维蛋白2(NF2)基因和merlin蛋白的上游和下游,和一些相应的目标,包括受体蛋白酪氨酸激酶(RTK),血管内皮生长因子受体(VEGFR),哺乳动物雷帕霉素靶复合物1(mTORC1)和血小板衍生生长因子受体(PDGFR)。在一些研究中已经报道了相当多的药物可以抑制VS细胞的增殖。大多数研究仍处于体外细胞实验和/或动物实验阶段。少数研究已进入Ⅰ期和Ⅱ期临床试验,但尚未导致任何临床治疗。本文对VS的药物治疗现状和前景进行了全面的了解,有利于后续研究的开展。
    Vestibular schwannoma (VS) is one of the most common types of benign tumors of the central nervous system. At present, the prevailing treatment methods of VS include surgery, stereotactic radiotherapy, and follow-up observation, etc. However, there is still no drug therapy available for treating VS. Although the surgical technique is relatively mature, the complications cannot be completely avoided. Furthermore, both the growth rate of different cases and patients\' sensitivity to radiotherapy vary greatly. With the constant progress made in molecular biology research, most of the studies on the growth mechanism of VS focus on the upstream and downstream of neurofibromin 2 ( NF2) gene and merlin protein, and a number of corresponding targets, including receptor protein tyrosine kinase (RTK), vascular endothelial growth factor receptor (VEGFR), mammalian target of rapamycin complex 1 (mTORC1) and platelet derived growth factor receptor (PDGFR). It has been reported in some studies that quite a few drugs could inhibit the proliferation of VS cells. Most of the studies are still in the stage of in vitro cell experiment and/or animal experiment. A small number of studies have entered phase Ⅰ and phase Ⅱ clinical trials, but have not led to any clinical treatment yet. This paper provides a comprehensive understanding of the current status and the prospects of drug therapies of VS, which is conducive to the development of subsequent research.
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  • 文章类型: Published Erratum
    [这更正了文章DOI:10.3389/fonc.2021.774462。].
    [This corrects the article DOI: 10.3389/fonc.2021.774462.].
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  • 文章类型: Journal Article
    UNASSIGNED:我们旨在探讨神经下切除技术在前庭神经鞘瘤(VSs)手术中分离和切除神经鞘膜下肿瘤的应用和前景。
    UNASSIGNED:这项研究回顾性分析了2015年7月至2020年10月在神经外科接受乙状窦后入路手术的124例VSs患者,唐都医院,空军军医大学.将就以下方面讨论数据:临床特征,手术策略,肿瘤切除范围,面部声学功能保护,术后并发症。
    UNASSIGNED:104例患者实现了肿瘤的大体全切除(GTR),GTR率为83.9%,20例患者获得肿瘤次全切除(STR)。GTR和STR在面神经和听神经功能保留方面无显著差异,以及在实体和囊性肿瘤之间的肿瘤切除。在完全解剖保留面神经方面,保留率达到97.6%。使用House-Brackmann(HB)分级评分评估面神经功能。因此,HB等级为I-II,III-IV,和V-VI测定为96(77.4%),25(20.2%),和3例(2.4%),分别,术后1周,占110例(88.7%),13例(10.5%),1例(0.8%),分别,在6个月。手术前听力可用的35例患者中,有15例(42.9%)在术后6个月仍具有听力可用。术后小脑或脑干出血5例,一名患者死亡。多因素logistic回归分析显示年龄较大(≥60岁,p=0.011),大肿瘤(>3厘米,p=0.004),囊性肿瘤(p=0.046)是与肿瘤与脑干和面听神经粘连程度相关的独立危险因素。
    UNASSIGNED:我们成功地将神经下切除技术应用于一系列VSs患者,并取得了令人满意的结果。准确识别会阴部及神经下切除肿瘤,可有效减少术中面听神经的扰动,为判断肿瘤边界提供直观依据。在VSs的手术治疗中,神经下切除技术可能有助于提高肿瘤的全切除率和面听神经功能的保留。
    UNASSIGNED: We aimed to explore the application and prospects of the subperineural resection technique for tumor separation and removal under the perineurium during surgery for vestibular schwannomas (VSs).
    UNASSIGNED: This study retrospectively analyzed 124 patients with VSs who underwent surgery via a retrosigmoid approach from July 2015 to October 2020 in the Department of Neurosurgery, Tangdu Hospital, Air Force Military Medical University. The data will be discussed with regard to the following aspects: clinical features, surgical strategies, tumor resection extent, facial-acoustic function preservation, and postoperative complications.
    UNASSIGNED: Gross total resection (GTR) of the tumor was achieved in 104 patients, with a GTR rate of 83.9%, and subtotal resection (STR) of the tumor was achieved in 20 patients. There was no significant difference in facial and acoustic nerve functional preservation between GTR and STR, as well as in tumor resection between solid and cystic tumors. The retention rate reached 97.6% in terms of complete anatomical facial nerve preservation. Facial nerve function was assessed using the House-Brackmann (HB) grading score. Consequently, HB grades of I-II, III-IV, and V-VI were determined for 96 (77.4%), 25 (20.2%), and 3 (2.4%) cases, respectively, 1 week postoperatively and accounted for 110 cases (88.7%), 13 cases (10.5%), and 1 case (0.8%), respectively, at 6 months. Fifteen of 35 (42.9%) patients with serviceable hearing before the operation still had serviceable hearing at 6 months postoperatively. There were 5 cases of cerebellar or brainstem bleeding after the operation, and one patient died. Multivariate logistic regression analysis showed that older age (≥60 years, p = 0.011), large tumor (>3 cm, p = 0.004), and cystic tumor (p = 0.046) were independent risk factors associated with the extent of adhesion between the tumor and the brainstem and facial-acoustic nerve.
    UNASSIGNED: We successfully applied the subperineural resection technique to a large series of patients with VSs and achieved satisfactory results. Accurate identification of the perineurium and subperineural resection of the tumor can effectively reduce the disturbance of the facial-acoustic nerve during the operation and provide an intuitive basis for judging the tumor boundary. The subperineural resection technique may be conducive to improving the rate of total tumor resection and facial-acoustic nerve functional preservation in the surgical treatment of VSs.
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  • 文章类型: Journal Article
    目的:探讨神经内镜辅助在经乙状窦后入路手术切除前庭神经鞘瘤的小管部分和随后的早期面神经转归中的作用。
    方法:在本研究中,回顾性分析了在单个机构进行乙状窦后清扫的前庭神经鞘瘤伴听管内延伸的患者。应用了几种手术技术来确保最大和安全地切除肿瘤。延伸到内部声道(IAC)小于10毫米的肿瘤被归类为A级,而那些延伸超过10mm的IAC被视为B级。在显微阶段结束时应用神经内镜来寻找B级肿瘤的潜在残留物。定义并测量绝对肿瘤延伸。House和Brackmann(HB)量表用于评估CNVII的即时结果。
    结果:在61例患者中,有38名女性和23名男性。共有18例(29.51%)为库斯二级,12例(19.67%)库斯三级,和31例(50.82%)Koos四级。A级38例(62.30%),B级23例(37.70%),60例(98.36%)大体全切除。在内窥镜可视化下检测到并完全清除了4例小管内残留物。比例明显更高(17%,p=0.02)在B级比A级的小管内残留物。在所有情况下,在解剖学上保留了CNVII和VIII。共有55例(90.16%)保留良好(HB1级和2级)面神经结果。
    结论:在B级前庭神经鞘瘤中,在最大程度的显微外科手术切除后,对听管内部分的内镜评估显示,17%的患者存在肿瘤残留.因此,建议对IAC(B级)内延伸超过10mm的肿瘤的潜在导管内残留进行内窥镜评估。
    OBJECTIVE: To explore the role of neuroendoscope assistance during surgical resection of the intracanalicular portion of vestibular schwannomas via the retrosigmoid approach and the subsequent early facial nerve outcomes.
    METHODS: Patients of vestibular schwannoma with intracanalicular extensions undergoing retrosigmoid dissection at a single institution were retrospectively analyzed in this study. Several surgical techniques were applied to ensure maximal and safe removal of tumors. Tumors extending less than 10 mm into the internal acoustic canal (IAC) were classified as Grade A, while those extending over 10 mm into IAC were taken as Grade B. Neuroendoscope was applied at the end of microscopic phase to search for potential remnants for Grade B tumors. Absolute tumor extension was defined and measured. House and Brackmann (HB) scale was used to evaluate immediate CN VII outcomes.
    RESULTS: Of the 61 patients, there were 38 females and 23 males. A total of 18 (29.51%) cases were Koos Grade II, 12 (19.67%) cases Koos Grade III, and 31 (50.82%) cases Koos Grade IV. There were 38 cases (62.30%) of Grade A and 23 cases (37.70%) of Grade B. Gross total resection was achieved in 60 cases (98.36%). Four cases of intracanalicular remnants were detected and completely removed under endoscopic visualizations. There was a significantly higher proportion (17%, p = 0.02) of intracanalicular remnants in Grade B than Grade A. CN VII and VIII were anatomically preserved in all cases. A total of 55 cases (90.16%) retained good (HB Grades 1 and 2) facial nerve outcomes.
    CONCLUSIONS: In Grade B vestibular schwannomas, after maximal microsurgical removal, endoscopic evaluation of the intracanalicular portion revealed residual tumors in 17% of the patients. Hence endoscopic evaluation of the potential intracanalicular remnants for tumor extending over 10 mm within IAC (Grade B) is recommended.
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  • 文章类型: Journal Article
    BACKGROUND: Vestibular schwannoma is the third most common benign intracranial tumor that can occur sporadically or be associated with neurofibromatosis type 2 (neurofibromatosis type 2 vestibular schwannoma [NF2-VS]). The aim of this study is to provide a comprehensive bioinformatic analysis of methylated-differentially expressed genes (MDEGs) in NF2-VS.
    METHODS: Transcriptional sequencing datasets (GSE141801 and GSE108524) and gene methylation microarrays (GSE56598) from the Gene Expression Omnibus database were used to identify and analyze MDEGs in NF2-VS. A protein-protein interaction (PPI) network was built, and the hub genes and modules were identified. Finally, potential pharmacotherapy targeting MDEGs were extracted for NF2-VS.
    RESULTS: A total of 57 hypermethylation-low expression genes and 88 hypomethylation-high expression genes were identified. Pathways associated with aberrantly MDEGs included P13K-AKT, MAPK, and Ras, which were also involved in NF2-VS. Six hub genes (EGFR, CCND1, CD53, CSF1R, PLAU, and FGFR1) were identified from the PPI network. Modification of the aforementioned genes altered cell-to-cell communication, response to stimulus, cellular regulation, and membrane and protein bindings. Thirty drugs targeting these pathways were selected based on the hub genes.
    CONCLUSIONS: Analysis of MDEGs may enrich the understanding of the molecular mechanisms of NF2-VS pathogenesis and lay the groundwork for potential biomarkers and therapeutic targets for NF2-VS.
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  • 文章类型: Systematic Review
    听力损失是散发性前庭神经鞘瘤(SVS)患者最常见的初始症状。保留听力是保守治疗和手术治疗的重要目标。然而,SVS相关听力损失的机制尚不清楚.因此,我们进行了这篇系统综述,以总结目前对SVS听力损失的理解,并提炼出一个可测试的假设,以进一步阐明其潜在的机制.
    查询四个数据库(PubMed,Medline,Embase,和WebofScience)进行了研究,以评估SVS患者的听力损失并探索听力障碍的潜在机制。
    共有50篇文章符合条件,并被纳入本评论。经过分析,检索到的研究可以分为四种类型:(1)29项研究探索听力损失和肿瘤生长模式之间的关系(例如,肿瘤大小/体积,增长率,肿瘤位置,等。);(2)十项研究调查了耳蜗功能障碍在听力恶化中的潜在作用,包括结构异常,外淋巴液中的蛋白质升高,和耳蜗故障;(3)两项研究调查了SVS引起的听觉通路和皮层损伤;(4)在其余九项研究中,研究人员探索了SVS听力损失的分子机制,涉及分子和遗传改变,炎症反应,生长因子,和其他肿瘤相关的分泌物.
    多种因素可能导致SVS听力受损,包括肿瘤的生长模式,耳蜗功能障碍,听觉通路和皮质受损,基因和分子的变化。然而,我们目前的理解仍然有限,需要未来的研究来探索这种多因素假设,并更深入地挖掘其潜在机制。
    UNASSIGNED: Hearing loss is the most common initial symptom in patients with sporadic vestibular schwannomas (SVS). Hearing preservation is an important goal of both conservative and surgical therapy. However, the mechanism of SVS-associated hearing loss remains unclear. Thus, we performed this systematic review to summarize the current understanding of hearing loss in the SVS and distill a testable hypothesis to further illuminate its underlying mechanism.
    UNASSIGNED: A systematic review querying four databases (PubMed, Medline, Embase, and Web of Science) was performed to identify studies evaluating hearing loss in patients with SVS and exploring the potential mechanisms of hearing impairment.
    UNASSIGNED: A total of 50 articles were eligible and included in this review. After analysis, the retrieved studies could be categorized into four types: (1) 29 studies explore the relationship between hearing loss and the growth pattern of the tumor (e.g., tumor size/volume, growth rate, tumor location, etc.); (2) ten studies investigate the potential role of cochlear dysfunction in hearing deterioration, including structural abnormality, protein elevation in perilymph, and cochlear malfunctioning; (3) two studies looked into SVS-induced impairment of auditory pathway and cortex; (4) in the rest nine studies, researchers explored the molecular mechanism underlying hearing loss in SVS, which involves molecular and genetic alterations, inflammatory response, growth factors, and other tumor-associated secretions.
    UNASSIGNED: Multiple factors may contribute to the hearing impairment in SVS, including the growth pattern of tumor, cochlear dysfunction, impairment of auditory pathway and cortex, genetic and molecular changes. However, our current understanding is still limited, and future studies are needed to explore this multifactorial hypothesis and dig deeper into its underlying mechanism.
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