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      Cancer-associated Nummular Loss of the Retinal Pigment Epithelium

      case-report

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          Abstract

          Purpose

          To report a case of cancer-associated nummular loss of the retinal pigment epithelium.

          Methods

          A 47-year-old man with a history of hepatocellular carcinoma presented with three weeks of bilateral visual loss. His best-corrected visual acuity was 20/40 in each eye. He had multiple round confluent grayish-brown patches at the level of retinal pigment epithelium, and no pigmented choroidal lesions. Fluorescein angiography showed circular areas of transmission defect and indocyanine green angiography showed early hyperfluorescence, corresponding with the multiple round confluent patches.

          Conclusions

          We report a case of visual paraneoplastic syndrome which showed nummular loss of the pigment epithelial cells which distinguishes the clinical component of BDUMP syndrome.

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          Most cited references9

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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).
          Article 0 comments Cited 31 times – based on 0 reviews     Review now
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            Paraneoplastic retinopathies and optic neuropathies.

            Jane W Chan (2015)
            Unusual neuro-ophthalmologic symptoms and signs that go unexplained should warrant a thorough investigation for paraneoplastic syndromes. Although these syndromes are rare, these clinical manifestations can herald an unsuspected, underlying malignancy that could be treated early and aggressively. This point underscores the importance of distinguishing and understanding the various, sometimes subtle, presentations of ocular paraneoplastic syndromes. Outlined in this review article are diagnostic features useful in differentiating cancer-associated retinopathy, melanoma-associated retinopathy, and paraneoplastic optic neuropathy. These must also be distinguished from non-cancer-related eye disorders that may clinically resemble cancer-associated retinopathy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Treatment outcomes from reported cases are summarized, and some potential novel immunotherapies are also discussed.
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              Bilateral diffuse uveal melanocytic proliferation in a patient with cancer-associated retinopathy.

              Wataru Saito, Hiroshi Ohguro, Masahiko Yokoi … (2005)
              To describe a patient with bilateral diffuse uveal melanocytic proliferation (BDUMP) and cancer-associated retinopathy (CAR). Interventional case report. A 66-year-old woman developed progressive vision loss 4 months after total hysterectomy. Ophthalmologic examination, Western blot test of sera and aqueous humor, and immunohistochemistry of carcinoma cells were performed. Testing revealed BDUMP and severe retinal dysfunction. Autoantibodies against recoverin and heat shock cognate protein 70 (hsc 70) were detected in serum. Cytoplasmic immunoreactivity for recoverin and hsc 70 was observed in endometrioid carcinoma cells. Simultaneous cases of BDUMP and CAR are rare. Aberrantly expressed recoverin and hsc 70 triggered serum autoantibody production, which caused photoreceptor degeneration.
              Article 0 comments Cited 13 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Korean J Ophthalmol
                Journal ID (publisher-id): KJO
                Title: Korean Journal of Ophthalmology : KJO
                Publisher: The Korean Ophthalmological Society
                ISSN (Print): 1011-8942
                Publication date (Print): December 2007
                Publication date (Electronic): 20 December 2007
                Volume: 21
                Issue: 4
                Pages: 261-264
                Affiliations
                Department of Ophthalmology, Gangnam Sacred Heart Hospital, College of Medicine, Hallym University, Seoul, Korea.
                Author notes
                Reprint requests to Ha Kyoung Kim, MD. Department of Ophthalmology, Kangnam Sacred Heart Hospital, College of Medicine, Hallym University, 948-1 Daerim-dong, Youngdeungpo-gu, Seoul, 150-950, Korea. Tel: 82-2-829-5193, Fax: 82-2-848-4638, hkkimeye@ 123456unitel.co.kr
                Article
                DOI: 10.3341/kjo.2007.21.4.261
                PMC ID: 2629894
                PubMed ID: 18063894
                SO-VID: 2532bd3e-3db0-4964-9abe-b0ba39b0fee3
                Copyright © Copyright © 2007 Korean Ophthalmological Society
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 03 May 2007
                Date accepted : 17 August 2007
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Ophthalmology & Optometry
                Keywords: bilateral diffuse uveal melanocytic proliferation,cancer-associated retinopathy,paraneoplastic syndrome
                Data availability:
                ScienceOpen disciplines: Ophthalmology & Optometry
                Keywords: bilateral diffuse uveal melanocytic proliferation, cancer-associated retinopathy, paraneoplastic syndrome

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