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Neuro-oncology Jan 2020The level of evidence to provide treatment recommendations for vestibular schwannoma is low compared with other intracranial neoplasms. Therefore, the vestibular...
The level of evidence to provide treatment recommendations for vestibular schwannoma is low compared with other intracranial neoplasms. Therefore, the vestibular schwannoma task force of the European Association of Neuro-Oncology assessed the data available in the literature and composed a set of recommendations for health care professionals. The radiological diagnosis of vestibular schwannoma is made by magnetic resonance imaging. Histological verification of the diagnosis is not always required. Current treatment options include observation, surgical resection, fractionated radiotherapy, and radiosurgery. The choice of treatment depends on clinical presentation, tumor size, and expertise of the treating center. In small tumors, observation has to be weighed against radiosurgery, in large tumors surgical decompression is mandatory, potentially followed by fractionated radiotherapy or radiosurgery. Except for bevacizumab in neurofibromatosis type 2, there is no role for pharmacotherapy.
Topics: Humans; Neuroma, Acoustic
PubMed: 31504802
DOI: 10.1093/neuonc/noz153 -
Head and Neck Pathology Dec 2020Vestibular schwannoma (VS) is a Schwann cell-derived tumour arising from the vestibulocochlear nerve. Although benign, it represents a threat to intracranial structures... (Review)
Review
Vestibular schwannoma (VS) is a Schwann cell-derived tumour arising from the vestibulocochlear nerve. Although benign, it represents a threat to intracranial structures due to mass effect and carries a small risk of malignant transformation. VS therefore represents an important healthcare burden. We review the literature regarding pathogenesis, risk factors, and diagnosis of VS. The current and future potential management strategies are also discussed. A narrative review of all relevant papers known to the authors was conducted. The majority of VS remain clinically stable and do not require interventional procedures. Nevertheless, various surgical techniques exist for removing VS, the most common of which are translabyrinthine and retrosigmoid approaches. Due to surgical risks such as hearing loss, facial nerve dysfunction, post-operative headache, and cerebrospinal fluid leakage, a "watch and rescan" approach is adopted for most patients. Radiotherapy is a useful alternative and has been shown to have a similar response for growth restriction. Due to the heterogeneous nature of VS, there is a lack of consensus regarding management of tumours that are too large for conservative management but too small to indicate surgery. Emerging biologic therapies, such as Bevacizumab, Everolimus, and Lapatinib, as well as anti-inflammatories like aspirin are promising potential treatments; however, long-term evidence of their efficacy is required. The knowledge base regarding VS continues to improve. With increased understanding of the pathogenesis of these tumors, we believe future work should focus on pharmacologic intervention. Biologic therapies aimed toward improved patient outcomes are particularly promising.
Topics: Humans; Neuroma, Acoustic; Risk Factors
PubMed: 32232723
DOI: 10.1007/s12105-020-01155-x -
International Journal of Molecular... May 2022Vestibular schwannoma (VS) is a benign tumor that originates from Schwann cells in the vestibular component. Surgical treatment for VS has gradually declined over the... (Review)
Review
Vestibular schwannoma (VS) is a benign tumor that originates from Schwann cells in the vestibular component. Surgical treatment for VS has gradually declined over the past few decades, especially for small tumors. Gamma knife radiosurgery has become an accepted treatment for VS, with a high rate of tumor control. For neurofibromatosis type 2 (NF2)-associated VS resistant to radiotherapy, vascular endothelial growth factor (VEGF)-A/VEGF receptor (VEGFR)-targeted therapy (e.g., bevacizumab) may become the first-line therapy. Recently, a clinical trial using a VEGFR1/2 peptide vaccine was also conducted in patients with progressive NF2-associated schwannomas, which was the first immunotherapeutic approach for NF2 patients. Targeted therapies for the gene product of SH3PXD2A-HTRA1 fusion may be effective for sporadic VS. Several protein kinase inhibitors could be supportive to prevent tumor progression because merlin inhibits signaling by tyrosine receptor kinases and the activation of downstream pathways, including the Ras/Raf/MEK/ERK and PI3K/Akt/mTORC1 pathways. Tumor-microenvironment-targeted therapy may be supportive for the mainstays of management. The tumor-associated macrophage is the major component of immunosuppressive cells in schwannomas. Here, we present a critical overview of targeted therapies for VS. Multimodal therapy is required to manage patients with refractory VS.
Topics: High-Temperature Requirement A Serine Peptidase 1; Humans; Neurilemmoma; Neurofibromatosis 2; Neuroma, Acoustic; Phosphatidylinositol 3-Kinases; Tumor Microenvironment; Vascular Endothelial Growth Factor A
PubMed: 35628268
DOI: 10.3390/ijms23105462 -
JAMA Aug 2023Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been... (Randomized Controlled Trial)
Randomized Controlled Trial
IMPORTANCE
Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically.
OBJECTIVE
To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma.
DESIGN, SETTING, AND PARTICIPANTS
Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021.
INTERVENTIONS
Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires.
MAIN OUTCOMES AND MEASURES
The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications.
RESULTS
Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed.
CONCLUSION AND RELEVANCE
Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT02249572.
Topics: Female; Humans; Male; Middle Aged; Neuroma, Acoustic; Radiosurgery; Retrospective Studies; Treatment Outcome; Watchful Waiting; Magnetic Resonance Imaging; Cerebellopontine Angle; Salvage Therapy; Microsurgery
PubMed: 37526718
DOI: 10.1001/jama.2023.12222 -
Yonsei Medical Journal May 2016Many epidemiological studies have investigated environmental risk factors for the development of acoustic neuroma. However, these results are controversial. We conducted... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Many epidemiological studies have investigated environmental risk factors for the development of acoustic neuroma. However, these results are controversial. We conducted a meta-analysis of case-control studies to identify any potential relationship between history of noise exposure, smoking, allergic diseases, and risk of acoustic neuroma.
MATERIALS AND METHODS
We searched PubMed to identify relevant articles. Two researchers evaluated the eligibility and extracted the data independently.
RESULTS
Eleven case-control studies were included in our meta-analysis. Acoustic neuroma was found to be associated with leisure noise exposure [odds ratio (OR)=1.33, 95% confidence interval (CI): 1.05-1.68], but not with occupational noise exposure and ever noise exposure (OR=1.20, 95% CI: 0.84-1.72 and OR=1.15, 95% CI: 0.80-1.65). The OR of acoustic neuroma for ever (versus never) smoking was 0.53 (95% CI: 0.30-0.94), while the subgroup analysis indicated ORs of 0.95 (95% CI: 0.81-1.10) and 0.49 (95% CI: 0.41-0.59) for ex-smoker and current smoker respectively. The ORs for asthma, eczema, and seasonal rhinitis were 0.98 (95% CI: 0.80-1.18), 0.91 (95% CI: 0.76-1.09), and 1.52 (95% CI: 0.90-2.54), respectively.
CONCLUSION
Our meta-analysis is suggestive of an elevated risk of acoustic neuroma among individuals who were ever exposed to leisure noise, but not to occupational noise. Our study also indicated a lower acoustic neuroma risk among ever and current cigarette smokers than never smokers, while there was no significant relationship for ex-smokers. No significant associations were found between acoustic neuroma and history of any allergic diseases, such as asthma, eczema, and seasonal rhinitis.
Topics: Adult; Asthma; Environmental Exposure; Female; Humans; Hypersensitivity; Leisure Activities; Neuroma, Acoustic; Noise; Occupational Exposure; Risk Factors; Smoking
PubMed: 26996581
DOI: 10.3349/ymj.2016.57.3.776 -
Wiener Medizinische Wochenschrift (1946) Feb 2022Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in...
Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in this context. Varying growth rates of these benign tumors complicate a prediction of functional impairment of cranial nerves. Therefore, a regular update on current therapeutic strategies and alternative treatment options is relevant for both physicians and patients.
Topics: Aftercare; Humans; Neuroma, Acoustic; Quality of Life
PubMed: 33439379
DOI: 10.1007/s10354-020-00800-y -
Neurology India 2022Hearing preservation in vestibular schwannoma resection is a much-discussed topic in these times of functional preservation. Irrespective of the mode of treatment of... (Review)
Review
BACKGROUND
Hearing preservation in vestibular schwannoma resection is a much-discussed topic in these times of functional preservation. Irrespective of the mode of treatment of large tumors, this becomes possible only in a lucky few. The overall burden of such deaf patients is on a rising trend, and therefore, it is high time neurosurgeons look into this often-neglected area of hearing rehabilitation.
OBJECTIVES
In this review, the authors intend to provide a glimpse of available hearing rehabilitation modalities in the present era.
MATERIALS AND METHODS
: This review deals with both immediate and long-term hearing outcomes following both surgery and stereotactic radiosurgery for sporadic vestibular schwannoma and the need for hearing rehabilitation in these patients. Various databases, including PubMed, Medline/Medscape, Scopus, Cochrane, and Google Scholar were searched to find available literature on hearing rehabilitation after treatment of sporadic vestibular schwannoma. The searched MeSH terms were "Hearing", "Vestibular schwannoma", "Rehabilitation", and "Acoustic Neuroma", alone and in various combinations.
RESULTS
: The authors were surprised to find a paucity of literature. Even though neurosurgeons are at the forefront in managing vestibular schwannoma, they are not actively involved in hearing rehabilitation techniques possible for those who are rendered deaf following treatment.
CONCLUSIONS
: The authors strongly believe that this review will be a wake-up call for neurosurgeons on a relatively untouched topic.
Topics: Hearing; Hearing Tests; Humans; Neuroma, Acoustic; Radiosurgery; Treatment Outcome
PubMed: 35864608
DOI: 10.4103/0028-3886.349600 -
World Neurosurgery Sep 2022Vestibular schwannomas are benign, slow-growing tumors that often reduce patient quality of life by compressing nearby nerves. Neurological function preservation is one... (Review)
Review
OBJECTIVE
Vestibular schwannomas are benign, slow-growing tumors that often reduce patient quality of life by compressing nearby nerves. Neurological function preservation is one of the indicators of treatment success, with hearing preservation being the most difficult to obtain. This paper provides a bibliometric analysis of hearing preservation in treating acoustic neuromas and a greater understanding of the most highly cited articles, which have enhanced our understanding of this topic.
METHODS
Key terms of "acoustic neuroma," "vestibular schwannoma," and "hearing preservation" were queried through Web of Science. Articles were sorted by citation frequency, and the top 100 articles were recorded for title, name of first author, journal title, year of publication, total number of citations (and associated rank), average number of citations per year, country of the first author's associated institution, and type of study.
RESULTS
The top 100 cited articles were published from 1980 to 2014. The United States had the highest involvement as a country (55%), the University of Pittsburgh as an institution (13%), and The Journal of Neurosurgery as a publishing source (27%). Fourteen were reviews, and 86 were clinical papers. Of the 86, 73 were retrospective studies.
CONCLUSION
Bibliometric analyses summarize and assess potential areas of strength and knowledge gaps within the literature. Studies on hearing preservation in vestibular schwannomas mostly consist of retrospective reviews that assess postoperative outcomes of microsurgery and radiosurgery. Prospective studies and novel treatment options for hearing preservation in vestibular schwannomas are needed to increase current literature diversity.
Topics: Hearing; Humans; Neuroma, Acoustic; Prospective Studies; Quality of Life; Radiosurgery; Retrospective Studies
PubMed: 35779753
DOI: 10.1016/j.wneu.2022.06.120 -
AJNR. American Journal of Neuroradiology Nov 2017Vestibular schwannomas are the most common cerebellopontine angle tumor. During the past century, the management goals of vestibular schwannomas have shifted from total... (Review)
Review
Vestibular schwannomas are the most common cerebellopontine angle tumor. During the past century, the management goals of vestibular schwannomas have shifted from total resection to functional preservation. Current treatment options include surgical resection, stereotactic radiosurgery, and observation. Imaging has become a crucial part of the initial screening, evaluation, and follow-up assessment of vestibular schwannomas. Recognizing and understanding the management objectives, various treatment modalities, expected posttreatment findings, and complications allows the radiologist to play an essential role in a multidisciplinary team by providing key findings relevant to treatment planning and outcome assessment. The authors provide a comprehensive discussion of the surgical management, role of radiation therapy and observation, imaging differential, and pre- and posttreatment imaging findings of vestibular schwannomas.
Topics: Cerebellar Neoplasms; Cerebellopontine Angle; Humans; Neuroma, Acoustic; Neurosurgical Procedures; Treatment Outcome
PubMed: 28546250
DOI: 10.3174/ajnr.A5213 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Dec 2022This study was conducted to evaluate the applications of vestibular function tests in diagnosis, identifying tumor origins and prognosis of vestibular rehabilitation of...
This study was conducted to evaluate the applications of vestibular function tests in diagnosis, identifying tumor origins and prognosis of vestibular rehabilitation of patients with acoustic neuroma. This research is a single-center cross-sectional clinical study, which retrospectively analyzed the data of 335 patients with acoustic neuroma from March 2013 to March 2020 in the Eye and ENT Hospital of Fudan University. The study included caloric test, cervical and ocular vestibular evoked myogenic potentials(cVEMP, oVEMP), video head impulse test(vHIT) and sensory organization test(SOT). Firstly, the sensitivity, specificity, and Yoden index of each test were calculated. Secondly, the internal relevance of these tests was studied for application in judging the origins of the tumor. The abnormal rates of caloric test, cVEMP, oVEMP, vHIT and SOT was 85.3%, 86.1%, 85.5%, 55.6% and 67.7% in these participants. Among all the vestibular function tests included, the caloric test showed the best sensitivity(0.855), specificity(0.981), and Yoden index(0.836). The study found that the higher the Koos grades, the higher the abnormal rates of the caloric test, vHIT, and oVEMP(Cochran-Armitage test, <0.05). There was no significant relationship between the combination of abnormal vestibular function tests and tumor origin nerves(>0.05). Majorlty of the participants in this study with acoustic neuroma showed abnormal results in SOT related to poor balance control. More than half of the patients had at least two abnormal result of the battery of vestibular function tests, among which the caloric test was proved to have better sensitivity and specificity. The higher the Koos grades of the tumor, the higher the abnormal rates of the caloric test, vHIT, and oVEMP.
Topics: Humans; Neuroma, Acoustic; Retrospective Studies; Cross-Sectional Studies; Vestibular Function Tests; Caloric Tests; Vestibular Evoked Myogenic Potentials; Head Impulse Test
PubMed: 36543397
DOI: 10.13201/j.issn.2096-7993.2022.12.004