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      Bilateral Diffuse Uveal Melanocytic Proliferation Presenting as Small Choroidal Melanoma

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          Abstract

          Purpose. To describe a patient with Bilateral Diffuse Uveal Proliferation who presented initially with a clinical picture consistent with choroidal melanoma. Methods. Presentation of a clinical case with fundus photos, fluorescein angiography, and optical coherence tomography. Results. A 70-year-old Caucasian male with history of esophageal cancer presented with an asymptomatic pigmented choroidal lesion in his left eye initially diagnosed as choroidal nevus. This lesion enlarged over the course of a year and developed orange pigment and increased thickness. A metastatic workup was negative, and a radioactive iodine plaque was placed on the left eye. Over the next six months, the visual acuity in his left eye decreased. His clinical picture was consistent with unilateral Diffuse Uveal Proliferation. A recurrence of his esophageal carcinoma with metastasis was discovered and palliative chemotherapy was initiated. Although his visual acuity improved in the left eye, similar pigmentary changes developed in the right fundus. His visual acuity in both eyes gradually decreased to 20/200 until his death a year later. Conclusion. BDUMP should always be considered in the differential diagnosis of patients with pigmented fundus lesions and a history of nonocular tumors.

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          Most cited references4

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          Bilateral diffuse uveal melanocytic proliferation in patients with occult carcinoma.

          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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            [On the pathogenesis of the flat malignant melanoma].

            R Machemer (1965)
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              Cancer-associated nummular loss of the pigment epithelium.

              To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) that had areas of retinal pigment epithelial loss. Observational case report. A 67-year-old woman with a history of uterine cancer presented with 4 months of bilateral visual loss. Although B-scan ultrasonography revealed both small shallow serous retinal and choroidal detachments in the periphery, the choroid was normal in thickness. Fluorescein angiography revealed numerous nummular-shaped areas of transmission defects suggesting retinal pigment epithelium loss. Autofluorescence photography showed complete absence of autofluorescence in these nummular areas, and optical coherence tomography showed segmental areas with lack of any signal from affected areas of the retinal pigment epithelium, suggesting complete loss of retinal pigment epithelium cells. Although the fundus findings in BDUMP have been attributed to the proliferation of uveal melanocytic cells in the outer choroid in previous papers, our patient had nummular areas of loss of the retinal pigment epithelium as the apparent reason for visual decline.
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                Author and article information

                Journal
                Case Rep Ophthalmol Med
                Case Rep Ophthalmol Med
                CRIM.OPHMED
                Case Reports in Ophthalmological Medicine
                Hindawi Publishing Corporation
                2090-6722
                2090-6730
                2011
                20 December 2011
                : 2011
                : 740640
                Affiliations
                Department of Ophthalmology, University of North Carolina at Chapel Hill, CB 7040, Chapel Hill, NC 27599, USA
                Author notes

                Academic Editors: K. K. Nischal and E. B. Rodrigues

                Article
                10.1155/2011/740640
                3350172
                22606473
                e7641886-e66a-4f49-a024-a2bd9c9a2c42
                Copyright © 2011 J. N. Ulrich et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 26 October 2011
                : 15 November 2011
                Categories
                Case Report

                Ophthalmology & Optometry
                Ophthalmology & Optometry

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