Neurological Surgery最新文献

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[Latest Classification and Management of Circumscribed Astrocytic Gliomas]. 局限性星形细胞胶质瘤的最新分类与治疗
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040764
Tomohiro Yamasaki, Kazuhiko Kurozumi
{"title":"[Latest Classification and Management of Circumscribed Astrocytic Gliomas].","authors":"Tomohiro Yamasaki, Kazuhiko Kurozumi","doi":"10.11477/mf.030126030540040764","DOIUrl":"10.11477/mf.030126030540040764","url":null,"abstract":"<p><p>The 2021 World Health Organization (WHO) classification of tumors of the central nervous system (CNS5) marks a major shift toward molecularly integrated diagnostics, distinguishing circumscribed astrocytic gliomas and glioneuronal tumors from diffuse gliomas based on characteristic genomic and epigenetic signatures. These tumors are predominantly driven by MAPK pathway alterations, including BRAF fusions and BRAF V600E mutation, whereas others harbor mTOR pathway activation or distinct drivers, such as PRKCA and MN1. Advances in precision oncology have transformed clinical management, and targeted therapies, such as dabrafenib/trametinib and everolimus, have been established; selumetinib received expanded adult approval in Japan in 2025. Moreover, next-generation type II RAF inhibitors, exemplified by tovorafenib, and emerging combinatorial approaches targeting pathway crosstalk offer promising strategies to overcome therapeutic resistance. This review synthesizes current molecular definitions and evolving personalized treatment paradigms for circumscribed gliomas. Integrating these molecular insights into routine practice is essential for optimizing tumor control and preserving neurological function in the CNS5 era.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"764-773"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148764840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Molecular Targeted Therapies Effective for Brain Metastases and Their Position in Guidelines]. [分子靶向治疗脑转移瘤的有效性及其在指南中的地位]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040822
Toshihiko Iuchi
{"title":"[Molecular Targeted Therapies Effective for Brain Metastases and Their Position in Guidelines].","authors":"Toshihiko Iuchi","doi":"10.11477/mf.030126030540040822","DOIUrl":"10.11477/mf.030126030540040822","url":null,"abstract":"<p><p>Various driver gene mutations have been identified, and agents targeting these molecular abnormalities have been developed and are being used in treatment. Some of these driver gene abnormalities are also associated with a high frequency of brain metastases. In patients with non-small cell lung cancer (NSCLC), more than 80% of brain metastases had a molecular target. Tyrosine kinase inhibitors are highly effective against brain metastases in patients with epidermal growth factor receptor-mutated or anaplastic lymphoma kinase fusion-positive NSCLC. Recently, the efficacy of the antibody-drug conjugate, T-DXd, against HER2+ breast cancer has also been confirmed. Many other molecularly targeted therapies are also effective against brain metastases; thus, many patients with brain metastases may benefit from these treatments. Although molecularly targeted therapy has become an important treatment modality for brain metastases, its limitations, such as not being curative and having a limited duration of effect, are also becoming clear. In the era of precision medicine, it is important to coordinate conventional local treatment with individually tailored molecularly targeted therapy. For that purpose, it is essential to understand the efficacy and limitations of targeted therapy for brain metastasis for each molecular target.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"822-832"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148761846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Cancer Genomic Medicine in Neuro-Oncology Practice]. [神经肿瘤实践中的癌症基因组医学]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040855
Makoto Ohno, Yoshitaka Narita
{"title":"[Cancer Genomic Medicine in Neuro-Oncology Practice].","authors":"Makoto Ohno, Yoshitaka Narita","doi":"10.11477/mf.030126030540040855","DOIUrl":"10.11477/mf.030126030540040855","url":null,"abstract":"<p><p>Cancer genomic medicine is an integral component of neuro-oncology, enabling personalized treatments based on tumor-specific genomic alterations. In Japan, comprehensive genomic profiling (CGP) is performed using several tissue- and plasma-based platforms to detect genomic mutations, copy number alterations, gene fusions, tumor mutational burden, and other molecular biomarkers. Tissue-based assays are preferred for brain tumors because of the limited sensitivity of plasma testing. CGP results were interpreted by expert molecular tumor boards supported by the Center for Cancer Genomics and Advanced Therapeutics (C-CAT), integrating genomic and clinical data and providing information on potential targeted therapies and clinical trials. Real-world studies have demonstrated that actionable alterations can be identified in most patients, although only a few patients ultimately receive genomically matched treatments. However, the selected patients may achieve meaningful clinical benefits. Currently, approved molecularly targeted therapies in neuro-oncology include vorasidenib, dabrafenib plus trametinib, entrectinib or larotrectinib, and pembrolizumab for IDH1/2-mutant gliomas, BRAF V600E-mutant gliomas, NTRK fusion-positive tumors, and TMB-high tumors, respectively. Future challenges include improving patient access to genomic medicine, optimizing the timing of testing, expanding genomic analyses, and facilitating the development of novel targeted therapies for rare brain tumors.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"855-863"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148766203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Role of Cerebral Hemodynamic and Metabolic Measurements in the Management of Ischemic Cerebrovascular Disease]. [脑血流动力学和代谢测量在缺血性脑血管病治疗中的作用]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040891
Yoji Tanaka
{"title":"[Role of Cerebral Hemodynamic and Metabolic Measurements in the Management of Ischemic Cerebrovascular Disease].","authors":"Yoji Tanaka","doi":"10.11477/mf.030126030540040891","DOIUrl":"10.11477/mf.030126030540040891","url":null,"abstract":"<p><p>Understanding cerebral hemodynamics and metabolism is essential for the diagnosis and treatment of ischemic cerebrovascular disease. Advances in imaging modalities and techniques have enabled the quantitative assessment of hemodynamic compromise and facilitated tailored treatment. In acute ischemic stroke, computed tomography/magnetic resonance perfusion imaging supports the identification of salvageable tissue (the penumbra) and contributes to the selection of endovascular thrombectomy (EVT), particularly within the delayed time window. In chronic intracranial atherosclerotic disease (ICAD), quantitative assessments of cerebral blood flow (CBF), cerebrovascular reactivity (CVR), and oxygen extraction fraction (OEF) are useful for evaluating the risk of recurrence. The results of clinical trials for extracranial-intracranial bypass surgery vary due to differences in selection criteria and perioperative risks; therefore, patient selection based on rigorous hemodynamic evaluation is crucial. Repeated hemodynamic assessments are helpful in determining treatment strategies in moyamoya disease, for instance, which is progressive, even in asymptomatic cases. Postoperative cerebral hemodynamics may present with a mixture of hyperperfusion and hypoperfusion. To reduce the risk of postoperative complications, it is key to accurately understand the hemodynamic status. Collectively, the measurement of cerebral hemodynamics and metabolism is crucial for individualized and evidence-based management of ischemic cerebrovascular disease.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"891-906"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148760704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Molecular Classification and Treatment of Ependymomas]. 室管膜瘤的分子分类与治疗
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040781
Joji Ishida, Shuichiro Hirano, Yoshihiro Otani, Shota Tanaka
{"title":"[Molecular Classification and Treatment of Ependymomas].","authors":"Joji Ishida, Shuichiro Hirano, Yoshihiro Otani, Shota Tanaka","doi":"10.11477/mf.030126030540040781","DOIUrl":"https://doi.org/10.11477/mf.030126030540040781","url":null,"abstract":"<p><p>Recent advances in molecular profiling have revealed that ependymomas are biologically distinct entities with unique molecular alterations and clinical behaviors. These findings have been incorporated into the 2021 WHO Classification of Tumors of the Central Nervous System, integrating anatomical location and molecular features into the diagnostic framework. This review summarizes the current molecular classification of ependymomas, role of DNA methylation profiling in diagnosis, and biological and clinical characteristics of the major molecular subgroups. Additionally, we discuss current treatment strategies, molecular risk stratification, and future directions in the management of ependymomas.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"781-788"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Treatment of von Hippel-Lindau Disease and Hemangioblastoma]. von Hippel-Lindau病和血管母细胞瘤的治疗
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040811
Shunsaku Takayanagi
{"title":"[Treatment of von Hippel-Lindau Disease and Hemangioblastoma].","authors":"Shunsaku Takayanagi","doi":"10.11477/mf.030126030540040811","DOIUrl":"10.11477/mf.030126030540040811","url":null,"abstract":"<p><p>Von Hippel-Lindau (VHL) disease is a hereditary tumor syndrome characterized by the development of neoplasms in multiple organs. Hemangioblastoma is one of the most common tumors associated with VHL disease. In 2024, the \"Clinical Practice Guidelines for VHL Disease\" were established in Japan to provide guidance for the diagnosis, treatment, and management of VHL disease. Furthermore, in 2025, the HIF-2αinhibitor belzutifan was approved for insurance coverage in Japan for VHL-associated tumors, including hemangioblastomas. In this article, we first provide an overview of VHL disease and the \"Clinical Practice Guidelines for VHL Disease,\" and then discuss the diagnosis and treatment of hemangioblastomas, including surgery, endovascular therapy, and radiotherapy. Finally, we review the role of the HIF-2αinhibitor belzutifan. In the future, management of VHL disease and hemangioblastomas will likely require not only individual treatment modalities, but also optimal multimodal strategies combining surgery, radiotherapy, and systemic drug therapy.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"811-821"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148759575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Recent Advances in the Treatment of Craniopharyngioma:A Breakthrough in Molecular Targeted Therapy]. [颅咽管瘤治疗新进展:分子靶向治疗的突破]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040844
Shingo Fujio, Ryosuke Hanaya
{"title":"[Recent Advances in the Treatment of Craniopharyngioma:A Breakthrough in Molecular Targeted Therapy].","authors":"Shingo Fujio, Ryosuke Hanaya","doi":"10.11477/mf.030126030540040844","DOIUrl":"10.11477/mf.030126030540040844","url":null,"abstract":"<p><p>Craniopharyngiomas are benign sellar and parasellar tumors that present significant clinical challenge because of their close proximity to the optic apparatus, hypothalamus, and pituitary gland. Recent molecular studies have identified distinct driver mutations, namely, CTNNB1 in adamantinomatous craniopharyngiomas and BRAF V600E in papillary craniopharyngiomas, leading to a deeper understanding of tumor biology and treatment strategies. Advances in endoscopic endonasal transsphenoidal surgery have improved surgical safety and expanded the indications for minimally invasive resection, whereas radiotherapy remains an effective option for residual or recurrent disease. Molecular targeted therapy with BRAF and MEK inhibitors has resulted in remarkable tumor shrinkage in papillary craniopharyngiomas harboring the BRAF V600E mutation, and recent clinical studies have established this approach as a promising treatment option. These agents have emerged not only as effective treatment options for recurrent or refractory disease but also as promising options for neoadjuvant treatment and function-preserving management. Future treatment strategies are likely to incorporate molecular subtypes, patient age, and functional outcomes to achieve individualized care. In addition, the development of reliable preoperative molecular diagnostic techniques, including liquid biopsy, may further facilitate the integration of targeted therapies into routine clinical practice.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"844-853"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148765293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Evolution of Glioma Diagnosis: The WHO 2021 Classification and Beyond]. [胶质瘤诊断的演变:WHO 2021分类及以后]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040716
Takashi Komori
{"title":"[Evolution of Glioma Diagnosis: The WHO 2021 Classification and Beyond].","authors":"Takashi Komori","doi":"10.11477/mf.030126030540040716","DOIUrl":"10.11477/mf.030126030540040716","url":null,"abstract":"<p><p>The World Health Organization (WHO) classification of brain tumors, which is based on the histogenetic concept, underwent a major transformation in the revised fourth edition published in 2016. Molecular genetic information was incorporated into the classification system, enabling the distinction of tumors based on the presence or absence of IDH mutations and 1p/19q co-deletion. This represents a significant paradigm shift in glioma diagnosis. In the fifth edition (WHO CNS5), the classification evolved by clearly separating adult- and paediatric-type gliomas and integrating genetic information directly into tumor grading. Molecular alterations are essential components of diagnosis. Consequently, the diagnostic criteria were refined to better reflect biological behavior and clinical outcomes. The forthcoming sixth edition may further advance this molecularly driven approach. Simultaneously, efforts are underway to establish a more coherent framework for using newly identified tumor entities and emerging molecular terminology. Thus, the WHO classification continues to evolve toward a more biologically meaningful and clinically relevant system for glioma diagnosis.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"716-725"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148766512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Treatment of Lower-Grade Glioma:Emergence of IDH Inhibitors and Changes in Therapeutic Decision-Making]. [低级别胶质瘤的治疗:IDH抑制剂的出现和治疗决策的变化]。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040747
Yohei Mineharu
{"title":"[Treatment of Lower-Grade Glioma:Emergence of IDH Inhibitors and Changes in Therapeutic Decision-Making].","authors":"Yohei Mineharu","doi":"10.11477/mf.030126030540040747","DOIUrl":"10.11477/mf.030126030540040747","url":null,"abstract":"<p><p>Lower-grade gliomas comprise a biologically heterogeneous group of adult diffuse gliomas, primarily defined by their molecular alterations. In current practice, management should integrate maximal safe resection, individualized use of radiotherapy, and rational systemic therapy based on the tumor subtype, growth pattern, and anticipated long-term functional outcomes. Surgery remains the cornerstone of treatment because the extent of resection influences tumor control, seizure burden, functional outcomes, and timing of adjuvant therapy. Although radiotherapy is effective, its timing, dose, and field design should be optimized to preserve neurocognitive function and quality of life in patients with prolonged life expectancy. Combined chemoradiotherapy remains essential for select high-risk tumors, whereas temozolomide alone cannot be considered a universal substitute for radiotherapy-based strategies. The emergence of IDH inhibitors, particularly vorasidenib, has expanded therapeutic options for select patients with residual or recurrent non-enhancing grade 2 IDH-mutant gliomas following surgery by prolonging progression-free survival and delaying the need for subsequent intervention, without apparent deterioration in health-related quality of life or neurocognition. This review summarizes current evidence and proposes a practical framework for integrating surgery, radiotherapy, conventional chemotherapy, and IDH inhibition into routine clinical decision-making.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"747-753"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148759237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Molecular Classification and Treatment of Medulloblastoma: An Update]. 髓母细胞瘤的分子分类和治疗:最新进展。
Neurological Surgery Pub Date : 2026-07-01 DOI: 10.11477/mf.030126030540040774
Kohei Fukuoka
{"title":"[Molecular Classification and Treatment of Medulloblastoma: An Update].","authors":"Kohei Fukuoka","doi":"10.11477/mf.030126030540040774","DOIUrl":"10.11477/mf.030126030540040774","url":null,"abstract":"<p><p>Medulloblastoma is a prevalent malignant pediatric central nervous system tumor. While medulloblastoma was historically risk-stratified by clinical factors, the WHO 2021 classification integrated molecular profiling, fundamentally transforming its diagnosis and management. The tumor is categorized into four primary molecular subgroups using techniques such as DNA methylation profiling: WNT-activated, SHH-activated, and non-WNT/non-SHH (Groups 3 and 4). WNT-activated tumors generally exhibit the most favorable prognosis. SHH-activated tumors present varying outcomes depending on patient age and genetic alterations, such as <i>TP53</i> mutations. Group 3 tumors, especially those with <i>MYC</i> amplification, frequently metastasize and have poor outcomes, whereas Group 4 tumors show a more prolonged clinical course. Standard treatment involves maximal safe resection, craniospinal irradiation (CSI), and chemotherapy. However, to mitigate severe late complications, molecular subtyping now enables precision and risk-adapted therapies. This includes exploring CSI dose reduction for the favorable WNT subgroup and utilizing novel molecular biomarkers for Groups 3 and 4. For infants, treatment primarily relies on chemotherapy to avoid CSI. Furthermore, recurrence patterns strongly correlate with these molecular subtypes. Re-biopsy and molecular re-evaluation are crucial upon relapse to distinguish genuine recurrences from radiation-induced secondary tumors.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"54 4","pages":"774-780"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762226","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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