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      Hemorrhagic vestibular schwannoma: a case example of vestibular apoplexy syndrome. Illustrative case

      case-report

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          Abstract

          BACKGROUND

          Acute intratumoral hemorrhage within a vestibular schwannoma, or vestibular apoplexy, is a rare condition. Unlike the typical insidious vestibulopathy typically caused by vestibular schwannoma growth, patients with vestibular apoplexy have an acute and severe presentation with nausea and emesis in addition to severe vertigo and hearing loss. Here, the authors present an illustrative case demonstrating this rare clinical condition and an operative video detailing the surgical management.

          OBSERVATIONS

          A 76-year-old man presented to the emergency department with acute-onset dizziness, left-ear fullness, double vision, gait ataxia, emesis, and facial numbness. Imaging revealed a 2.8-cm hemorrhagic left cerebellopontine angle lesion extending into the left internal auditory canal, consistent with hemorrhagic vestibular schwannoma. The patient subsequently underwent a retrosigmoid craniotomy for resection of the hemorrhagic mass, and by 1 month after surgery, all his presenting symptoms had resolved, allowing his return to daily activities.

          LESSONS

          Vestibular schwannomas typically present with decreased hearing and chronic vestibulopathy. Acute presentation should raise the suspicion for an apoplectic event, and surgical debulking may lead to improvement in most vestibular symptoms.

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          Most cited references36

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          EANO guideline on the diagnosis and treatment of vestibular schwannoma

          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.
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            Spontaneous intracranial meningioma bleeding: clinicopathological features and outcome.

            The aim of this study was to determine the clinicopathological features of patients with intracranial bleeding from unsuspected meningioma and to relate these data to surgery-related outcome. The authors report on two cases in which hemorrhage of an unsuspected meningioma occurred in the tentorial ridge and in the falx, and they discuss the details of 143 cases described in the literature. A bleeding propensity index of the meningioma, related to the patient's age, sex, and the lesion's intracranial location and histological type was computed as a ratio between the frequencies of bleeding meningioma and all meningiomas. This was tested by independent samples t-test for proportions. A chi-square test was used to determine the correlations between several variables: location and type of bleeding; survival and type of bleeding; and consciousness and survival. Increased bleeding tendency was found to be associated with two age groups ( 70 years), convexity and intraventricular locations, and fibrous meningiomas. The overall mortality rate documented in cases of bleeding meningiomas was 21.1% (13.9% in the computerized tomography [CT] scanning era), and that in surgically treated cases was 9.5% (7.5% in the CT scanning era). The overall major morbidity rate was 36% (33.8% in the CT scanning era). Overall 96.2% of conscious patients survived after their meningiomas spontaneously hemorrhaged. In patients who were unconscious before surgery, overall mortality rate was 74.1%, and that in surgically treated cases was 46.2%. The mortality rate in preoperatively conscious patients (those in whom acute deterioration and irreversible brain damage were prevented by early diagnosis and definitive surgery) was similar (< 3% in the CT scanning era) to that documented in cases in which meningiomas did not bleed. In contrast, the associated morbidity rates were much higher. One-stage total removal of the hemorrhagic meningioma and hematoma is the treatment of choice in such patients.
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              The natural history of vestibular schwannoma.

              The incidence of vestibular schwannomas (VSs) approaches 20 per million/yr. As treatment may depend on tumor growth, there is a demand of a treatment strategy based on hard data on the growth pattern of these tumors. This article reports growth data registered prospectively in 552 patients. Of the 1,818 consecutive patients, diagnosed with VS during the period from 1975 to 2005, 729 patients were allocated to observation by repetitive magnetic resonance imaging. At least two scans had been performed in 552 patients at the time of data analysis. Two hundred thirty patients had a tumor confined to the internal acoustic meatus, whereas 322 patients had a tumor with an extrameatal extension. Growth to extrameatal extension was the definition for growth in intrameatal tumors, whereas a largest diameter change of more than 2 mm was the criteria for growth/shrinkage of extrameatal tumors. The mean observation time was 3.6 years (range, 1-15 yr). Seventeen percent of the intrameatal tumors grew, whereas significantly more of the extrameatal tumors displayed growth during the period (28.9%). Growth occurred within the first 5 years after diagnosis. No correlation could be demonstrated between tumor growth rate, sex, or age. VS growth occurs within the first 5 years after diagnosis in a limited number of tumors, primarily in tumors with an extrameatal extension. We found no relation between tumor growth and sex or age. These findings justify primary observation of small tumors. A treatment strategy is proposed for this disease, focusing on the patient group allocated to observation.
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                Author and article information

                Journal
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                Journal of Neurosurgery: Case Lessons
                American Association of Neurological Surgeons
                2694-1902
                4 April 2022
                4 April 2022
                : 3
                : 14
                : CASE21722
                Affiliations
                [1]Department of Neurological Surgery, University of California, San Francisco, San Francisco, California
                Author notes
                Correspondence Lauro N. Avalos: University of California, San Francisco, San Francisco, CA. lauro.avalos@ 123456ucsf.edu .

                INCLUDE WHEN CITING Published April 4, 2022; DOI: 10.3171/CASE21722.

                Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

                Article
                CASE21722
                10.3171/CASE21722
                9379692
                bb3c9802-43b5-4478-b169-3e0c52efd8b6
                © 2022 The authors

                CC BY-NC-ND 4.0 ( http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 20 January 2022
                : 10 February 2022
                Page count
                Figures: 4, Tables: 0, References: 36, Pages: 4
                Categories
                Oncology, Oncology
                Vestibular Schwannoma, Vestibular Schwannoma
                Case Lesson

                vestibular apoplexy,vestibular schwannoma,hemorrhagic,dbhl = decibels hearing level,iac = internal auditory canal,mri = magnetic resonance imaging

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