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      Bilateral Diffuse Uveal Melanocytic Proliferation Presenting as a Giant Unilateral Choroidal Nevus: A Case Report

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          Abstract

          Background/Aims: The aim of our study was to report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) with a markedly asymmetric presentation and fundoscopic response to palliative chemotherapy. Case Report: We report a 67-year-old Caucasian man who presented with vision loss in his right eye. The best-corrected visual acuities were 2/10 in the right eye and 10/10 in the left eye, and biomicroscopy revealed bilateral mild cataracts. Fundoscopy of the right eye showed a macular flat and pigmented lesion extending beyond the posterior pole with areas of giraffe-type pigmentation and an overlying exudative retinal detachment. Nothing remarkable was detected in the left eye apart from a small round hypopigmented area of retinal pigment epithelium atrophy in the papillomacular bundle. BDUMP was diagnosed, and the workup for systemic malignancy revealed a pulmonary adenocarcinoma. After chemotherapy, not only did the right eye's visual acuity improve and the serous detachment resolve, but also the pigmentation decreased. Conclusion: BDUMP presentation can be markedly asymmetric and resemble a giant unilateral choroidal nevus. Response to chemotherapy was unique not only for the usual retinal detachment resolution, but also because of an evident regression of pigmentation.

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          Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: evaluation and management.

          Ehsan Rahimy, David Sarraf (2016)
          Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Timely recognition of one of these entities can facilitate early detection and treatment of an unsuspected, underlying malignancy, sometimes months before it would have otherwise presented, and gives the patient an increased chance at survival. We outline the clinical features, pathogenesis, and treatment strategies for the retinal- and optic nerve-based paraneoplastic syndromes: cancer-associated retinopathy; melanoma-associated retinopathy; paraneoplastic vitelliform maculopathy; bilateral diffuse uveal melanocytic proliferation; paraneoplastic optic neuropathy; and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Distinguishing these disorders from their non-paraneoplastic counterparts (e.g., autoimmune-related retinopathy and optic neuropathy, and acute zonal occult outer retinopathy) and determining appropriate systemic evaluation for the responsible tumor can be challenging. In addition, we discuss the utility and interpretation of autoantibody testing. Published by Elsevier Inc.
          Article 0 comments Cited 53 times – based on 0 reviews     Review now
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            Bilateral diffuse uveal melanocytic proliferation in patients with occult carcinoma.

            D Beahm, Stephen E. Pautler, Adam C. Wilkinson … (1990)
            The development of multiple, round or oval, subtle, red patches at the level of the pigment epithelium in the posterior ocular fundus and their striking early hyperfluorescence angiographically are characteristic features of the bilateral diffuse uveal melanocytic proliferation syndrome. They may be accompanied by severe visual loss and may antedate the appearance of multiple melanocytic tumors, retinal detachment, and cataract in these patients with occult systemic carcinomas. These hyperfluorescent patches are caused by focal damage to the pigment epithelium overlying an intact choriocapillaris and diffuse benign nonpigmented uveal melanocytic infiltration of the outer choroid. We suggest that outer retinal damage may not be primarily caused by melanocytic proliferation, but rather by toxic and immune factors generated by interaction between a systemic carcinoma and congenital melanocytosis of the uveal tract. We report the longest survivor of this disorder to date (102 months).
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              Bilateral Diffuse Uveal Melanocytic Proliferation With a Positive Ophthalmoscopic and Visual Response to Plasmapheresis

              Rebecca B. Mets (2011)
              Article 0 comments Cited 16 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (publisher-id): OOP
                Journal ID (pmc): OOP
                Journal ID (doi): 10.1159/issn.2296-4657
                Title: Ocular Oncology and Pathology
                Publisher: S. Karger AG
                ISSN (Print): 2296-4681
                ISSN (Electronic): 2296-4657
                Publication date Subscription-year: 2016
                Publication date (Print): September 2015
                Publication date (Electronic): 04 September 2015
                Volume: 2
                Issue: 1
                Pages: 54-58
                Affiliations
                aDepartment of Ophthalmology, Hospital Pedro Hispano, Matosinhos, bClínica Oftalmológica Dr. Neves Martins, Porto, and cHospital Privado da Trofa, Trofa, Portugal
                Author notes
                *Carlos Menezes, MD, Rua Arménio Reis, lote B4, PT-4700-267 Real, Braga (Portugal), E-Mail cafsousamenezes@gmail.com
                Article
                Publisher ID: 438944 Pmcid ID: PMC4847654 Other ID: Ocul Oncol Pathol 2016;2:54-58
                DOI: 10.1159/000438944
                PMC ID: PMC4847654
                PubMed ID: 27171585
                SO-VID: 960a6592-e267-4d8a-93bb-187174f16f54
                Copyright © © 2015 S. Karger AG, Basel
                License:

                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
                Date received : 01 June 2015
                Date accepted : 21 July 2015
                Page count
                Figures: 3, References: 15, Pages: 5
                Categories
                Subject: Case Series and Brief Reports

                ScienceOpen disciplines: Vision sciences,Ophthalmology & Optometry,Pathology
                Keywords: Bilateral diffuse uveal melanocytic proliferation,Paraneoplastic retinopathy,Chemotherapy
                Data availability:
                ScienceOpen disciplines: Vision sciences, Ophthalmology & Optometry, Pathology
                Keywords: Bilateral diffuse uveal melanocytic proliferation, Paraneoplastic retinopathy, Chemotherapy

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