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      Orbital Pseudocellulitis: A Retinoblastoma-Associated Masquerade Syndrome

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          Abstract

          Introduction: A masquerade syndrome is an atypical presentation of a neoplastic process that mimics an inflammatory condition. In this paper, we focus on orbital pseudocellulitis. Case Series: Our case series includes 5 retinoblastoma patients with orbital pseudocellulitis at presentation. In 3 patients the disease was bilateral, in 1 trilateral, and in 1 unilateral. The eyes with pseudocellulitis were enucleated, while the fellow eyes were treated conservatively, when affected. Four patients responded well to the therapy and showed remission of the tumor. The patient with trilateral retinoblastoma did not respond to therapy and died of disease. Discussion: Differential diagnosis with infectious orbital cellulitis is extremely important. Patients with orbital cellulitis present with fever, sinusitis, leukocytosis, and raised inflammatory markers, while ophthalmoscopic examination is negative and imaging studies show sinus involvement. On the contrary, patients with retinoblastoma do not show systemic inflammation, while ophthalmoscopic examination reveals leukocoria, buphthalmos, and an intraocular tumor mass associated with retinal detachment. Magnetic resonance imaging shows intralesional calcifications and soft tissue edema without sinus involvement. Histology confirms the diagnosis. Conclusions: Medical history, physical examination, and imaging studies are crucial in the diagnosis of retinoblastoma-associated orbital pseudocellulitis. Retinoblastoma should be excluded in all patients with signs of pre-septal orbital cellulitis through fundoscopy and/or imaging studies

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          Most cited references 22

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          Neoplastic Masquerade Syndromes

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            Retinoblastomas initially misdiagnosed as primary ocular inflammations.

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              Diffuse infiltrating retinoblastoma.

               R. Bhatnagar,  A Vine (1991)
              The authors describe two children who presented with atypical posterior uveitis and who were subsequently diagnosed as having diffuse infiltrating retinoblastoma. A review of these two cases and 26 previously published cases was performed to assess the epidemiology, presenting signs, and usefulness of diagnostic tests. In contrast to typical retinoblastoma, results of histologic analysis showed that only 4 of the 28 cases with infiltrating lesions contained calcium. Computed tomography scans and ultrasonography were not particularly helpful and many eyes required an anterior chamber paracentesis to establish the correct diagnosis. A systematic approach to the diagnosis of diffuse infiltrating retinoblastoma is presented.
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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
                2020
                December 2020
                08 October 2020
                : 6
                : 6
                : 430-437
                Affiliations
                aUnit of Ophthalmology, Department of Medicine, Surgery and Neuroscience, Azienda Ospedaliera Universitaria Senese, Siena, Italy
                bUnit of Neuroimaging and NeuroIntervention, Azienda Ospedaliera Universitaria Senese, Siena, Italy
                cUnit of Pathological Anatomy, Department of Medicine, Surgery, and Neurosciences, University of Siena, Siena, Italy
                Author notes
                *Maria Chiara Gelmi, Unit of Ophthalmology, Department of Medicine, Surgery and Neuroscience, Azienda Ospedaliera Universitaria Senese, Viale Mario Bracci 16, IL–53100 Siena (Italy), mariachiara.gelmi@student.unisi.it
                Article
                509810 Ocul Oncol Pathol 2020;6:430–437
                10.1159/000509810
                © 2020 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.

                Page count
                Figures: 3, Tables: 2, Pages: 8
                Categories
                Clinical Research

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