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      Ectopic cerebellar tissue in the occipital bone: a case report

      case-report

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          Abstract

          Background

          Ectopic cerebellar tissue located distantly from the normal cerebellum is very rare, and its pathophysiology remains to be elucidated.

          Case presentation

          We report an extremely rare case of intraosseous ectopic cerebellum detected incidentally at suboccipital craniotomy in a 46-year-old Japanese woman with hemifacial spasm. She had a small bone defect in the occipital bone, which contained a tiny area of soft tissue surrounded by cerebrospinal fluid connecting to the normal subarachnoid space through a dural opening. Histopathology demonstrated cerebellar cortex tissue consisting of molecular and granular cell layers.

          Conclusions

          This is the first report of glioneuronal ectopia within the skull bone separated from normal brain tissue, and it is important to distinguish this entity from other osteolytic lesions.

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          Most cited references23

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          Heterotopic glial nests in the subarachnoid space; histopathologic characteristics, mode of origin and relation to meningeal gliomas.

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            Primary diffuse multinodular leptomeningeal gliomatosis: case report and review of the literature.

            Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data with previously published observations. A 50-year-old man was admitted to the neurosurgery department for a previous 4-month history of headache, associated with nonspecific neurological signs. Biologic data and cerebrospinal fluid examination suggested an inflammatory process. The patient was given an antituberculous therapy. Magnetic resonance imaging revealed a multinodular enhancement of spinal nerve roots. A biopsy of sacral rootlets was performed. Histological examination revealed an anaplastic astrocytoma. Patient's status worsened, and death occurred 7 months later. Complete neuraxis postmortem examination revealed no intraparenchymatous glioma and was conclusive for the diagnosis of primary leptomeningeal gliomatosis (astrocytic, World Health Organization grade III), with a multinodular pattern in the spinal cord, the brainstem, and the brain base with diffuse extension into the cerebellar subarachnoid spaces. Our case illustrates the diagnostic difficulties in making the premortem diagnosis. The review of the literature indicates that there are no specific clinical or biologic signs. Magnetic resonance imaging using T1-weighted images with gadolinium enhancement and biopsy material may be useful diagnostic tools. In most cases, autopsy evaluation alone permits definitive primary diffuse leptomeningeal gliomatosis diagnosis. Whatever the histological characteristics of proliferating cells are, the prognosis remains poor. No prognostic factors have been shown to be correlated with survival time. Unfortunately, no routine treatment has been yet proposed.
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              Cerebellar heterotopia in the orbit.

              An infant with slowly progressive proptosis was found to have heterotopic cerebellum in her left orbit. Although cerebellar cell rests are common, to our knowledge none has been found previously in the orbit. It probably arose either from abnormal germ cell migration or from aberrant germ cell differentiation. Orbital heterotopia probably can be classified in the larger category of orbital encephaloceles because of similarities in presentation, clinical course, and treatment, although there can be differences in the embryologic mechanisms of their development.
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                Author and article information

                Contributors
                +81-492-76-1111 , mrkawashima-tky@umin.ac.jp
                mkob@saitama-med.ac.jp
                ishizawa@saitama-med.ac.jp
                tfujimaki@saitama-med.ac.jp
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                21 August 2017
                21 August 2017
                2017
                : 11
                : 231
                Affiliations
                [1 ]ISNI 0000 0004 0640 5017, GRID grid.430047.4, Department of Neurosurgery, , Saitama Medical University Hospital, ; 38 Morohongo, Moroyama-machi, Iruma, Saitama 350-0495 Japan
                [2 ]ISNI 0000 0004 0640 5017, GRID grid.430047.4, Department of Pathology, , Saitama Medical University Hospital, ; Saitama, Japan
                Article
                1394
                10.1186/s13256-017-1394-0
                5563901
                28823247
                e1ccb909-7570-4d01-ab2a-cd8d170f922b
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 31 May 2017
                : 19 July 2017
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2017

                Medicine
                ectopic cerebellum,glioneuronal ectopia,intraosseous lesion,occipital bone
                Medicine
                ectopic cerebellum, glioneuronal ectopia, intraosseous lesion, occipital bone

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