Journal
SURVEY OF OPHTHALMOLOGY
Volume 58, Issue 5, Pages 430-458Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.survophthal.2012.09.001
Keywords
paraneoplastic; cancer-associated retinopathy; melanoma-associated retinopathy; vitelliform maculopathy; bilateral diffuse uveal melanocytic proliferation; optic neuropathy; POEMS syndrome; autoimmune-related retinopathy and optic neuropathy; anti-retinal antibody; autoantibody testing
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Funding
- Karl Kirchgessner Foundation at the Jules Stein Eye Institute
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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.
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