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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 -
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 -
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 -
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 -
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 -
The Journal of International Advanced... Jan 2023Few investigations have been conducted on the clinical characteristics of the differential diagnosis of acoustic neuroma with acute sensorineural hearing loss and...
BACKGROUND
Few investigations have been conducted on the clinical characteristics of the differential diagnosis of acoustic neuroma with acute sensorineural hearing loss and idiopathic sudden sensorineural hearing loss. The aim of the study was to investigate the clinical characteristics of the differential diagnoses between acoustic neuroma and idiopathic sudden sensorineural hearing loss.
METHODS
The medical records of patients with acute sensorineural hearing loss (142 ears), including acoustic neuroma (19 ears) and idiopathic sudden sensorineural hearing loss (123 ears), who underwent audiometric and hematologic examinations and received systemic corticosteroid treatment, were retrospectively reviewed.
RESULTS
Hematological examination revealed that the erythrocyte sedimentation rate and fibrinogen values were significantly higher in the idiopathic sudden sensorineural hearing loss group compared to the acoustic neuroma group. Although all patients received corticosteroid treatment, hearing thresholds at the initial examination and 3 months after corticosteroid treatment were significantly higher in the idiopathic sudden sensorineural hearing loss group compared to the acoustic neuroma group at all frequencies. However, hearing recovery was worse in the acoustic neuroma group compared to the idiopathic sudden sensorineural hearing loss group. Furthermore, speech discrimination and short increment sensitivity index tests were not significantly different between the acoustic neuroma and idiopathic sudden sensorineural hearing loss groups.
CONCLUSION
This is the first study to reveal that speech discrimination and short increment sensitivity index tests are not useful for the differential diagnoses between acoustic neuroma and idiopathic sudden sensorineural hearing loss, whereas erythrocyte sedimentation rate and fibrinogen, blood biomarkers of inflammation and blood viscosity, would be considered valuable. Furthermore, acoustic neuroma should be considered in cases where acute sensorineural hearing loss did not recover after corticosteroid treatment, although the initial hearing loss was mild.
Topics: Humans; Neuroma, Acoustic; Retrospective Studies; Hearing Loss, Sensorineural; Hearing Loss, Sudden; Adrenal Cortex Hormones; Fibrinogen
PubMed: 36718029
DOI: 10.5152/iao.2023.22720 -
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 -
Journal of Clinical Neuroscience :... May 2022The incidence of acoustic neuromas in the United States is 1.09 per 100,000 with 23,739 newly diagnosed cases in the years 2004 to 2010. Because the recent literature...
The incidence of acoustic neuromas in the United States is 1.09 per 100,000 with 23,739 newly diagnosed cases in the years 2004 to 2010. Because the recent literature has supported that frailty can serve as a more accurate predictor of patient outcomes when evaluated with age, and is an important variable to consider in the course of patient treatment. The objective of this study was to compare the outcomes of frail patients who had undergone surgery for acoustic neuroma with their non-frail counterparts.The authors conducted a retrospective cohort study of geriatric patients receiving cranial neurosurgery for acoustic neuroma between 2016 and 2017 by using the Nationwide Readmission Database. A total of 396 frail patients and 402 non-frail patients were identified through the database of undergoing surgery for acoustic neuroma. Frail patients had statistically higher rates of readmission (p < 0.01), post-operative infection (p < 0.01), facial paralysis (p < 0.01), urinary tract infection (p < 0.01), hydrocephalus (p < 0.01), and dysphagia (p < 0.01). These post-op morbidities likely led to the increased length of stay (p < 0.01), non-routine discharge (p < 0.01), and all payer cost seen in frail patients (p < 0.01). However, no significant difference was found between frail and non-frail patients with regards to CSF leak, post hemorrhagic anemia, myocardial infarction, and mortality. Patient frailty status is a significant predictor of poor outcomes in the postoperative sequelae of acoustic neuroma surgery. Further, models including patient frailty plus age outperformed those using age alone for prediction of several postoperative complications.
Topics: Aged; Frail Elderly; Frailty; Humans; Length of Stay; Neuroma, Acoustic; Patient Readmission; Postoperative Complications; Retrospective Studies; Risk Factors; United States
PubMed: 35278933
DOI: 10.1016/j.jocn.2022.03.013 -
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 -
Mathematical Biosciences and... Jul 2022Acoustic neuroma is a common benign tumor that is frequently associated with postoperative complications such as facial nerve dysfunction, which greatly affects the...
Acoustic neuroma is a common benign tumor that is frequently associated with postoperative complications such as facial nerve dysfunction, which greatly affects the physical and mental health of patients. In this paper, clinical data of patients with acoustic neuroma treated with microsurgery by the same operator at Xiangya Hospital of Central South University from June 2018 to March 2020 are used as the study object. Machine learning and SMOTE-ENN techniques are used to accurately predict postoperative facial nerve function recovery, thus filling a gap in auxiliary diagnosis within the field of facial nerve treatment in acoustic neuroma. First, raw clinical data are processed and dependent variables are identified based on clinical context and data characteristics. Secondly, data balancing is corrected using the SMOTE-ENN technique. Finally, XGBoost is selected to construct a prediction model for patients' postoperative recovery, and is also compared with a total of four machine learning models, LR, SVM, CART, and RF. We find that XGBoost can most accurately predict the postoperative facial nerve function recovery, with a prediction accuracy of 90.0% and an AUC value of 0.90. CART, RF, and XGBoost can further select the more important preoperative indicators and provide therapeutic assistance to physicians, thereby improving the patient's postoperative recovery. The results show that machine learning and SMOTE-ENN techniques can handle complex clinical data and achieve accurate predictions.
Topics: Facial Nerve; Humans; Machine Learning; Neuroma, Acoustic; Postoperative Complications; Postoperative Period
PubMed: 36032000
DOI: 10.3934/mbe.2022487