{"title":"[Management of Neurofibromatosis Type 2].","authors":"Masazumi Fujii","doi":"10.11477/mf.030126030530040811","DOIUrl":"10.11477/mf.030126030530040811","url":null,"abstract":"<p><p>Surgical and radiotherapeutic interventions for vestibular schwannomas in patients with neurofibromatosis type 2 (NF2) generally yield less favorable outcomes than in sporadic unilateral cases, often hindering hearing preservation. Managing NF2 entails a lifelong series of complex decisions, repeatedly weighing functional preservation against impairment and surveillance against invasive therapeutic interventions. For both patients and their physicians, a critical issue lies in how to maximize functional preservation while simultaneously securing long-term survival. Auditory reconstruction using implantable auditory devices should be considered in cases of severe bilateral hearing loss. In patients with preserved cochlear nerve integrity, cochlear implantation is effective, and treatment strategies should be planned with this option in mind. Clinical trials of bevacizumab for pharmacological treatment are currently ongoing in Japan, and the clinical application of anti-vascular endothelial growth factor receptor vaccine therapy is anticipated.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"811-818"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883954","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}
{"title":"[Preoperative Neuroimaging of Cerebellopontine Angle Tumors with an Emphasis on Techniques and Differential Diagnosis].","authors":"Kazuhiro Tsuchiya","doi":"10.11477/mf.030126030530040618","DOIUrl":"10.11477/mf.030126030530040618","url":null,"abstract":"<p><p>MRI is the most effective imaging tool for diagnosing cerebellopontine angle tumors, although CT is also useful for evaluating bone changes and detecting calcification. Regarding MRI, it is recommended to efficiently use MR cisternography, a small imaging field of view, and a thin slice thickness. The most common tumor type is acoustic schwannoma, followed by meningioma, trigeminal, facial nerve, jugular foramen schwannoma, paraganglioma, and others. Many of these tumor types can be effectively differentiated by combining various CT and MRI techniques, as stated above, as well as MRA, perfusion imaging, MR digital subtraction angiography, MR spectroscopy, and bone imaging. This article discusses the key MRI and CT findings of major cerebellopontine angle tumors, as well as some representative cases and the corresponding differential diagnoses.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"618-625"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883961","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}
{"title":"[Surgical Strategy for Cerebellopontine Angle Epidermoid Cysts].","authors":"Hiroki Sakamoto, Michihiro Kohno","doi":"10.11477/mf.030126030530040763","DOIUrl":"10.11477/mf.030126030530040763","url":null,"abstract":"<p><p>Intracranial epidermoid cysts are congenital benign tumors; however, there is a long-term risk of recurrence if the tumor capsule is left behind perioperatively. Additionally, in cerebellopontine angle surgery, in which cranial nerves and blood vessels are densely concentrated, cranial nerve functions must be preserved besides radical removal of the tumor capsule. Particularly, the optimal surgical field should be obtained under direct visualization to avoid cranial nerve damage due to manipulation over cranial nerves and leaving the tumor behind in the blind spot of the surgical field. Therefore, besides the use of intraoperative monitoring, high surgical skills are required to use four skull base surgical approaches, such as lateral suboccipital retrosigmoid approach (LSO), anterior transpetrosal approach (ATP), combined transpetrosal approach, and ATP and LSO, depending on the size and progression of the tumor. Moreover, this disease should be treated at a specialized facility for skull base surgery.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"763-772"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883922","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}
{"title":"[Overview of Cerebellopontine Angle Tumors].","authors":"Michihiro Kohno","doi":"10.11477/mf.030126030530040612","DOIUrl":"10.11477/mf.030126030530040612","url":null,"abstract":"<p><p>Cerebellopontine angle tumors are rare, and surgeries for these tumors are challenging. Vestibular schwannomas, which account for 70-80% of cerebellopontine angle tumors, are managed by surgery, stereotactic radiosurgery or radiotherapy, and watchful observation. Surgery for vestibular schwannoma aims for maximal tumor resection while preserving facial and/or hearing function through strict intraoperative neuromonitoring. Surgical outcomes have markedly improved since the centralization of vestibular schwannoma surgery through the Internet and mass media. This chapter outlines the surgical approaches for cerebellopontine angle tumors, including vestibular schwannomas, meningiomas, epidermoid cysts, trigeminal schwannomas, jugular foramen schwannomas, facial nerve schwannomas, hypoglossal schwannomas, and glomus jugulare tumors.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"612-617"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883957","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}
{"title":"[Radiation Therapy for Cerebellopontine Angle Tumors:In the Standpoint of Gamma Knife Radiosurgery].","authors":"Yoshiyasu Iwai","doi":"10.11477/mf.030126030530040782","DOIUrl":"10.11477/mf.030126030530040782","url":null,"abstract":"<p><p>Stereotactic radiation therapy, including stereotactic radiosurgery, is a well-established and effective treatment for cerebellopontine angle tumors such as meningiomas, vestibular schwannomas, trigeminal and jugular foramen schwannomas, and glomus tumors. It offers high rates of tumor control while preserving neurological function, particularly in tumors smaller than 3 cm, which are ideal candidates for stereotactic radiosurgery. Large tumors or those extending beyond the skull base can also be managed effectively using fractionated stereotactic radiation therapy. As such, a multidisciplinary approach that combines surgical resection with stereotactic radiation therapy is a valuable strategy, especially when functional preservation is a key therapeutic goal.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"782-791"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883918","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}
{"title":"[Surgical Strategies for Trigeminal Schwannomas].","authors":"Shunsuke Shibao","doi":"10.11477/mf.030126030530040722","DOIUrl":"10.11477/mf.030126030530040722","url":null,"abstract":"<p><p>Trigeminal schwannomas are rare, benign tumors with diverse extension patterns along the trigeminal nerve, which significantly influence surgical approach selection. This article presents strategies for selecting optimal approaches based on tumor location, classified into middle, posterior, and extracranial compartments. Commonly used approaches include the anterior transpetrosal, epidural and interdural approaches, and recently introduced minimally invasive techniques, such as the endoscopic endonasal transmaxillary-pterygoid and transorbital approaches. The anatomical accessibility, safety, and feasibility of each technique are discussed. A detailed understanding of the trigeminal nerve's membranous anatomy, including the inner reticular layer and Meckel's cave, enables subcapsular dissection while preserving functional neural structures. This article describes practical techniques, including multilayer skull base reconstruction, venous preservation modifications, and intraoperative neurophysiological monitoring, to mitigate complications, such as venous injury, cerebrospinal fluid leakage, and cranial nerve dysfunction. Additionally, it offers a comprehensive overview of the decision-making and technical nuances essential for safe and effective resection of trigeminal schwannomas in complex skull base regions.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"722-737"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883921","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}
{"title":"[Transcranial and Endonasal Skull Base Approaches for Cerebello-Pontine Angle Lesions].","authors":"Hiroki Morisako, Atsufumi Nagahama, Takeo Goto","doi":"10.11477/mf.030126030530040682","DOIUrl":"10.11477/mf.030126030530040682","url":null,"abstract":"<p><p>Cerebellopontine angle lesions should be reduced as much as possible while preserving the cranial nerve and brainstem functions. However, because the lesion is located deep and surrounded by various important structures, surgical procedure is difficult to perform, and a surgical strategy is important to avoid complications. Surgical outcomes have dramatically improved with the development of skull bases and microsurgical techniques. The main surgical approaches for cerebellopontine angle lesions include the anterior and posterior combined transpetrosal, anterior transpetrosal, lateral suboccipital, and endoscopic endonasal approaches. With the recent developments in endoscopic keyhole surgery, such as the endoscopic keyhole anterior transpetrosal approach, minimally invasive surgery for skull-base lesions has gradually begun. Here, we describe the preoperative checkpoints, selection of surgical approaches, and surgical techniques for the resection of cerebellopontine angle lesions.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"682-692"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883923","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}
{"title":"[Molecular Pathogenesis, Genetic Profiles, and Therapeutic Strategies for NF2-Related Schwannomatosis].","authors":"Yu Teranishi, Satoru Miyawaki, Nobuhito Saito","doi":"10.11477/mf.030126030530040802","DOIUrl":"10.11477/mf.030126030530040802","url":null,"abstract":"<p><p>Neurofibromatosis type 2 (NF2), now redefined as NF2-related schwannomatosis (NF2-SWN), is a hereditary tumor syndrome characterized by bilateral vestibular schwannomas, multiple meningiomas, and other central nervous system tumors. The revised diagnostic criteria incorporate genetic testing and consideration of somatic mosaicism, enabling a more accurate and earlier diagnosis. A strong genotype-phenotype correlation has been established: truncating mutations are associated with an earlier onset and severe clinical course, whereas missense or splice-site mutations are often linked to milder phenotypes and better functional outcomes. Immune and molecular profiling have revealed distinct subtypes of vestibular schwannomas and meningiomas, reflecting differences in tumor behavior and therapeutic responses. In young patients presenting with unilateral vestibular schwannoma, clinicians should remain alert to the possibility of NF2-SWN and consider surveillance based on progression and genetic background. Targeted therapies-including bevacizumab, brigatinib, and vascular endothelial growth factor -A vaccines-have shown promise, particularly in refractory cases. Although routine intensive surveillance is not universally required, early genetic screening and long-term follow-ups may benefit select individuals. The comprehensive integration of genetic, pathological, and immunological data is essential for advancing personalized treatment strategies for NF2-SWN.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"802-810"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883956","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}
{"title":"[Plastic Surgical Reconstruction Treatments to Achieve Early and Maximal Improvement in Both Aesthetic and Functional Quality of Life for Patients with Facial Paralysis].","authors":"Yorikatsu Watanabe","doi":"10.11477/mf.030126030530040792","DOIUrl":"10.11477/mf.030126030530040792","url":null,"abstract":"<p><p>Recent advancements in reconstructive surgery for facial paralysis following cerebellopontine angle tumor resection have markedly enhanced early and maximal quality of life restoration. Contemporary management paradigms employ multidimensional strategies integrating functional and aesthetic rehabilitation, with hybrid approaches (e.g., static brow lift procedures combined with dynamic free muscle transfers) showing superior outcomes. The timing of referral to a facial paralysis specialist plastic surgeon from the onset of paralysis is critical for optimizing reconstructive outcomes and treatment selection; therefore, early consultation with neurosurgery is strongly recommended.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"792-801"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883958","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}
{"title":"[Surgical Management for Hypoglossal Schwannoma].","authors":"Yoichi Nonaka","doi":"10.11477/mf.030126030530040754","DOIUrl":"10.11477/mf.030126030530040754","url":null,"abstract":"<p><p>Hypoglossal schwannomas are extremely rare benign tumors arising from Schwann cells of the 12<sup>th</sup> cranial nerve. Surgical management of hypoglossal schwannomas requires detailed knowledge of the anatomy around the lower cerebellopontine angle (CPA), the craniovertebral junction (CVJ), and high cervical regions. If a tumor extends through the hypoglossal canal to the extracranial space, the canal is exposed by drilling the posterior half of the occipital condyle (OC), maintaining craniovertebral stability. Based on our experience and a literature review, we proposed the following modified grading scale to facilitate surgical planning: Type A, intradural tumors; Type B, dumbbell-shaped tumors; Type C, extracranial tumors; and Type D, peripheral tumors. The standard lateral suboccipital approach without opening the foramen magnum (FM) does not always provide adequate operative exposure for intradural type tumors. The transcondylar approach with FM opening and high cervical exposure allows gross total resection of dumbbell-shaped tumors. This specific skull base approach consists of an extended lateral suboccipital craniectomy with partial removal of the OC, which provides a wider view of the lower CPA without excessive cerebellar retraction. Key techniques leading to good outcomes are drilling of the OC, soft tissue dissection around the vertebral artery, and accurate exposure of the anterolateral CVJ.</p>","PeriodicalId":35984,"journal":{"name":"Neurological Surgery","volume":"53 4","pages":"754-762"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883920","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}