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      • Abstract: found
      • Article: found

      Primary Orbital Cystic Pilocytic Astrocytoma

      case-report

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          Abstract

          Purpose: To describe the clinical, radiological and histopathological features of a highly unusual primary orbital glioma. Procedures: A 6-year-old female presented with right-sided proptosis that revealed a well-defined intraconal mass on CT imaging with intact orbital bony walls. During the removal of the lesion, it dissected away freely from the muscles and was separated from the optic nerve and globe. There was no communication with the brain. Results: Histology showed histological features highly favouring a pilocytic astrocytoma enveloped by meninges. No non-neoplastic brain tissue was identified. Conclusion: This is the first documentation of a primary orbital pilocytic astrocytoma not anatomically connected with the brain. We speculate that the tumour had arisen from developmental brain heterotopia.

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          Most cited references8

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          Optic pathway gliomas in children with and without neurofibromatosis 1.

          Optic pathway gliomas represent 2 to 5% of brain tumors in children. Frequently asymptomatic, sometimes they demonstrate rapid growth, causing considerable visual dysfunction, neurologic deficits, and endocrine disturbances. Most optic pathway gliomas are diagnosed in patients with neurofibromatosis 1. Little is known about their natural course; therefore, there are no clear and widely accepted guidelines for their treatment. This study compared the clinical manifestations and natural history of sporadic and neurofibromatosis 1-associated optic pathway gliomas with regard to age at diagnosis, gender, and findings on neurologic, ophthalmologic, and neuroradiologic examinations in 83 children with optic pathway gliomas: 51 children with neurofibromatosis 1 and 32 children without any symptoms or signs of neurofibromatosis 1. A prospective study was performed in 21 patients with neurofibromatosis 1. In the rest of the patients with neurofibromatosis 1 and in 32 children with sporadic tumors, the analysis was carried out retrospectively. There was an increased incidence of females in the group of patients with neurofibromatosis 1 with optic pathway gliomas compared with the entire group of patients with neurofibromatosis 1 remaining for follow-up (P = .013). All optic pathway gliomas were found in children below 10 years of age, slightly earlier in the group without neurofibromatosis 1 (median age 4.6 vs 4.8 years). Children with optic pathway gliomas associated with neurofibromatosis 1 had predominantly multifocal lesions (P = .0001), whereas in the group without neurofibromatosis 1, isolated chiasmal involvement was more common (P = .002). Children with sporadic gliomas had significantly more frequently increased intracranial pressure, decreased visual acuity, and abnormalities of fundus of the eye at the time of diagnosis. The radiologic progression, visual deterioration, and endocrinologic complications were documented on follow-up more commonly in children with sporadic tumors. Our findings support the concept that there is an earlier and more severe clinical presentation of optic pathway gliomas in children with sporadic tumors than in those associated with neurofibromatosis 1.
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            Ectopic brain in the orbit.

            We report a case of ectopic brain tissue in the orbit associated with a bony defect of the orbit and the presence of skeletal muscle. Previous reports of orbital ectopic brain and related lesions in the orbit and at other sites are reviewed.
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              Ectopic brain tissue in the orbit.

              The authors report findings in a 9-month-old male infant with heterotopic brain tissue in the orbit, and compare and contrast the characteristics in this patient with the few other descriptions of such lesions in the literature.
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                Author and article information

                Journal
                OOP
                OOP
                10.1159/issn.2296-4657
                Ocular Oncology and Pathology
                S. Karger AG
                2296-4681
                2296-4657
                2016
                October 2016
                20 July 2016
                : 2
                : 4
                : 280-284
                Affiliations
                aNational Specialist Ophthalmic Pathology Service (NSOPS), Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK; bChittagong Eye Infirmary and Training Complex, Chittagong, Bangladesh
                Author notes
                *Dr. Hardeep Singh Mudhar, National Specialist Ophthalmic Pathology Service (NSOPS), Department of Histopathology, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF (UK), E-Mail hardeep.mudhar@sth.nhs.uk
                Article
                448006 PMC5091200 Ocul Oncol Pathol 2016;2:280-284
                10.1159/000448006
                PMC5091200
                27843910
                1a396edb-cb61-4391-bb23-af71672ec33d
                © 2016 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 13 January 2016
                : 23 June 2016
                Page count
                Figures: 2, References: 12, Pages: 5
                Categories
                Case Series and Brief Reports

                Vision sciences,Ophthalmology & Optometry,Pathology
                Brain heterotopia,Pilocytic astrocytoma,Orbit,Primary

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