Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management |
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Authors: | Ehsan Rahimy David Sarraf |
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Institution: | 1. Jules Stein Eye Institute, UCLA, Los Angeles, California, USA;2. Greater LA VA Healthcare Center, Los Angeles, California, USA |
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Abstract: | 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. |
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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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