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      Primary Lymphoma of Internal Acoustic Meatus Mimicking Vestibular Schwannoma—A Rare Diagnostic Dilemma

      case-report

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          Abstract

          Background/Setting  A subject presenting with a unilateral sensorineural hearing loss and with vertigo/imbalance and a lesion of internal acoustic meatus (IAM) most often represents a vestibular schwannoma. Several alternative pathologies involving the region, with clinical and neuroradiological similarities, could lead to an error in judgement and management. Rare tumors of the IAM pose unique diagnostic difficulty. A rare case that we present here had a typical history and imaging findings suggestive of vestibular schwannoma. A primary central nervous system (CNS) lymphoma was diagnosed in later stages of brain involvement warranting a retrospective analysis of the entity.

          Case Summary  An 80-year-old male presented with unilateral sensorineural hearing loss, vertigo, and imbalance. On imaging, he was found to have a lesion in the left internal auditory meatus, reported as a vestibular schwannoma and operated upon. Subject's condition worsened with time and a repeat imaging was suggestive of a CNS lymphoma with lesions involving bilateral cerebellum and subcortical white matrix.

          Conclusion  To conclude, primary CNS lymphoma presenting an isolated lesion in the IAM with no other parenchymal lesions at presentation is a rare incidence; to our knowledge this is the first case of such unique presentation.

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          Most cited references36

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          Age, gender, and racial differences in incidence and survival in primary CNS lymphoma

          Background: Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma that accounts for ∼4% of newly diagnosed central nervous system (CNS) tumours. The objective of this study was to analyse the epidemiology, incidence, and outcome of these rare tumours. Methods: Primary brain and CNS lymphoma cases were identified from the Surveillance, Epidemiology, and End Results (SEER) research data sets for the years 1980–2008 for analysis of trends in incidence and survival. SEER*Stat v. 7.0.4 software was used to analyse the data. Results: The overall incidence rate of PCNSL was 0.47 per 100 000 person-years. The incidence was significantly higher in males compared with females, blacks aged 0–49 years at diagnosis compared with whites, and whites aged 50 years and older at diagnosis compared with blacks. After a significant decline in incidence between 1995 and 1999, incidence rates rose slightly; those aged 75+ years at diagnosis had the most dramatic increase in incidence rates over time. Five-year survival rates were significantly higher in whites compared with blacks aged 0–49 years at diagnosis, but was primarily driven by white women aged 0–49 years. Conclusion: There is an increase in incidence of PCNSL in the elderly, and elderly blacks have lower incidence compared with white population. Survival remains poor and is negatively dominated by factors associated with HIV infection and advanced age.
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            Diagnosis and treatment of primary CNS lymphoma in immunocompetent patients: guidelines from the European Association for Neuro-Oncology.

            The management of primary CNS lymphoma is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the very few controlled studies available. In 2013, the European Association of Neuro-Oncology created a multidisciplinary task force to establish evidence-based guidelines for immunocompetent adults with primary CNS lymphoma. In this Review, we present these guidelines, which provide consensus considerations and recommendations for diagnosis, assessment, staging, and treatment of primary CNS lymphoma. Specifically, we address aspects of care related to surgery, systemic and intrathecal chemotherapy, intensive chemotherapy with autologous stem-cell transplantation, radiotherapy, intraocular manifestations, and management of elderly patients. The guidelines should aid clinicians in their daily practice and decision making, and serve as a basis for future investigations in neuro-oncology.
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              True incidence of vestibular schwannoma?

              The incidence of diagnosed sporadic unilateral vestibular schwannomas (VS) has increased, due primarily to more widespread access to magnetic resonance imaging. To present updated epidemiological data on VS incidence, as well as patient age, hearing acuity, tumor size, and localization at diagnosis for the last 4 decades in an unselected population, with emphasis on developments in recent years. From 1976 to 2008, 2283 new cases of VS were diagnosed and registered in a national database covering 5.0 to 5.5 million inhabitants. Incidence during the period, patient sex and age, data on hearing (pure tone average and speech discrimination), and tumor size at diagnosis were retrieved from the database. The incidence increased from 3.1 diagnosed VS per million per year in 1976 to a peak of 22.8 VS per million per year in 2004, which was followed by a decrease to 19.4 VS per million per year in 2008. Mean tumor size at diagnosis decreased from 30 mm in 1979 to 10 mm in 2008, whereas hearing acuity at diagnosis has improved over the years. After a steady increase over the last 4 decades, the incidence of vestibular schwannomas appears to have peaked and decreased in recent years, stabilizing at about 19 tumors per million per year. Whereas the sex ratio and age at diagnosis have remained grossly unchanged over the years, hearing has improved, and tumor size has decreased considerably.
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                Author and article information

                Journal
                J Neurol Surg Rep
                J Neurol Surg Rep
                10.1055/s-00000182
                Journal of Neurological Surgery Reports
                Georg Thieme Verlag KG (Rüdigerstraße 14, 70469 Stuttgart, Germany )
                2193-6358
                2193-6366
                January 2021
                23 February 2021
                : 82
                : 1
                : e1-e5
                Affiliations
                [1 ]Department of Otorhinolaryngology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra, India
                [2 ]Department of Radiology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra, India
                Author notes
                Address for correspondence Swati Kodur, MBBS, DLO Department of ENT, Dr. Balabhai Nanavati Hospital SV Road, Vile Parle West, Mumbai-56, MaharashtraIndia drswatikodur@ 123456gmail.com
                Author information
                http://orcid.org/0000-0003-4557-4588
                Article
                190017
                10.1055/s-0040-1722343
                7902110
                bdddfcf6-71d9-4834-887d-ad7f1ac292ca
                The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ )

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License, which permits unrestricted reproduction and distribution, for non-commercial purposes only; and use and reproduction, but not distribution, of adapted material for non-commercial purposes only, provided the original work is properly cited.

                History
                : 26 March 2019
                : 25 February 2020
                Categories
                Case Report

                vestibular schwannoma,internal acoustic meatus,cns lymphoma,imaging of internal acoustic meatus lesions

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