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      Diagnosis and management of bilateral vestibular schwannoma in the cerebellopontine angle: A rare case report

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          Abstract

          Vestibular Schwannoma (VS) is a benign nerve sheath tumors comprised of Schwann cells. This tumor is encapsulated, slow-growing, and originates from the internal auditory canal, extending into the cerebellopontine angle (CPA). The incidence in individuals aged 20-44 is 0.75 per 100,000 cases, with bilateral VS incidence of 0.8 per 50,000 cases. Tumors in CPA are the most common type in the posterior fossa and cause serious neurological symptoms or become life-threatening when tumors enlarge and compress the brainstem. The majority of tumors are VS (acoustic neuromas), accounting for 80%-90% of cases. Common clinical symptoms include hearing loss, tinnitus, and vertigo. Additionally, these tumors cause compression of the trigeminal and facial nerves. Advances in rapidly evolving imaging technology and surgical methods have improved diagnosis and management. A 24-year-old male complained of hearing impairment for the past 3 years alongside headaches, and dizziness leading to a feeling of imbalance, double, and blurry vision, as well as a sensation of facial thickness on the left side. Neurological examination showed cranial nerve abnormalities, including bilateral paresis of cranial nerves III, IV, VI, left cranial nerves V and VII, bilateral cranial nerve VIII, right cranial nerve XII, and cerebellar abnormalities such as intention tremor, dysmetria, dysdiadokokinesia, wide-based gait, and falling to the right during Romberg testing with both eyes open and closed. The patient underwent a contrast-enhanced MRI of the head, followed by a right CPA tumors excision through craniotomy. A detailed understanding of the medical history, physical examination, and radiological proved to be crucial in establishing an accurate diagnosis and appropriate management. This was considered essential to minimize a worse prognosis.

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          Most cited references10

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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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            Koos Classification of Vestibular Schwannomas: A Reliability Study.

            The Koos classification of vestibular schwannomas is designed to stratify tumors based on extrameatal extension and compression of the brainstem. While this classification system is widely reported in the literature, to date no study has assessed its reliability.
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              Peripheral tumor and tumor-like neurogenic lesions.

              Neoplasms of neurogenic origin account for about 12% of all benign and 8% of all malignant soft tissue neoplasms. Traumatic neuroma, Morton neuroma, lipomatosis of a nerve, nerve sheath ganglion, perineurioma, benign and malignant peripheral nerve sheath tumors (PNST) are included in this group of pathologies. Clinical and radiologic evaluation of patients with neurogenic tumors and pseudotumors often reveals distinctive features. In this context, advanced imaging techniques, especially ultrasound (US) and magnetic resonance (MR) play an important role in the characterization of these lesions. Imaging findings such as location of a soft tissue mass in the region of a major nerve, nerve entering or exiting the mass, fusiform shape, abnormalities of the muscle supplied by the nerve, split-fat sign, target sign and fascicular appearance should always evoke a peripheric nerve sheath neoplasm. Although no single imaging finding or combination of findings allows definitive differentiation between benign from malign peripheric neurogenic tumors, both US and MR imaging may show useful features that can lead us to a correct diagnosis and improve patient treatment. Traumatic neuromas and Morton neuromas are commonly associated to an amputation stump or are located in the intermetatarsal space. Lipomatosis of a nerve usually appears as a nerve enlargement, with thickened nerve fascicles, embedded in evenly distributed fat. Nerve sheath ganglion has a cystic appearance and commonly occurs at the level of the knee. Intraneural perineuroma usually affects young people and manifests as a focal and fusiform nerve enlargement. In this article, we review clinical characteristics and radiologic appearances of these neurogenic lesions, observing pathologic correlation, when possible.
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                Author and article information

                Contributors
                Journal
                Radiol Case Rep
                Radiol Case Rep
                Radiology Case Reports
                Elsevier
                1930-0433
                13 January 2024
                April 2024
                13 January 2024
                : 19
                : 4
                : 1271-1275
                Affiliations
                [0001]Department of Neurology, Neurology, Airlangga University Faculty of Medicine, Dr. Soetomo General Hospital, Mayjend Prof. Dr., Moestopo No. 6 - 8, Surabaya-60264, Surabaya-Indonesia
                Author notes
                [* ]Corresponding author. athalia.talaway@ 123456gmail.com
                Article
                S1930-0433(23)00956-1
                10.1016/j.radcr.2023.12.042
                10825540
                38292801
                6b42c2b2-6427-4006-a9af-1002831fb937
                © 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 5 October 2023
                : 20 December 2023
                : 21 December 2023
                Categories
                Case Report

                diagnosis,management,cpa tumors,bilateral vestibular schwannoma,acoustic neuroma

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