Paraneoplastic retinopathies: an update on pathogenesis, diagnosis and management

: The paraneoplastic retinopathies are a heterogeneous group of disorders with significant visual consequences occuring in the setting of a systemic malignancy. These conditions may be characterized by the presence of antiretinal antibodies and may predate or follow the diagnosis of an underlying malignancy. Herein I review the clinical findings, pathophysiology, laboratory testing and management of the paraneoplastic retinopathies: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), bilateral diffuse uveal melanocytic proliferation (BDUMP) and paraneoplastic vitelliform maculopathy (PVM). The pathophysiology of the paraneoplastic retinopathies varies from molecular mimicry resulting in anti-retinal antibody production (CAR, MAR) to relases of soluble factors either by the primary tumor (BDUMP) and/or immune system in response to the primary tumor (PVM) which result in retinal and/or retinal pigment epithelium dysfunction. For each condition, structural and functional multimodal retinal testing can be helpful in establishing the diagnosis. Treatment for the paraneoplastic retinopathies involves a combination of treating the underlying malignancy plus additional local and/or systemic immunosuppressive agents though no systemic therapeutic protocols exist. Despite these interventions, the retinopathy may be progressive or the retinopathy may be a harbinger of poor survival. Nevertheless, prompt diagnosis may help identify an underlying malignancy earlier and may thus improve cancer-related survival.