Paraneoplastic disorders can affect any part of the nervous system. Neurologists, oncologists, and ophthalmologists need to be aware that this includes the retinal and optic pathways. Retinal degeneration may occur in association with a number of systemic neoplasms, including--most notably--melanoma and small cell lung carcinoma. For many patients, it is vision loss that is the presenting feature of the associated tumor. Some affected patients have circulating antibodies against retinal antigens; these antibodies serve as diagnostic markers for the condition and may also play a role in causing retinal dysfunction. In this article, the author summarizes the clinical features, autoimmune pathogenesis, and treatment options for patients with paraneoplastic retinal degeneration.
• Paraneoplastic retinopathy is a rare complication of a variety of neoplasms, most commonly small cell lung carcinoma or melanoma.
• In most patients with carcinoma-associated paraneoplastic retinopathy, subacute vision loss is the presenting feature of the tumor, whereas the majority of patients with melanoma-associated paraneoplastic retinopathy develop vision loss after the melanoma diagnosis.
• Most patients with paraneoplastic retinopathy have one or more antiretinal autoantibodies with varying immunohistochemical staining patterns and specificity for a number of retinal antigens.
• Immunosuppressive therapy leads to improved vision in many patients with paraneoplastic retinopathy, so early diagnosis may lead to better outcome.
Historical note and terminology
The first well-documented cases of "photoreceptor degeneration as a remote effect of cancer" were reported in 1976 by Sawyer and colleagues (Sawyer et al 1976). The term "paraneoplastic retinopathy" encompasses patients with heterogeneous tumor associations and clinical features and probably represents more than 1 pathophysiologic mechanism.
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