Paraneoplastic syndromes and the retina

Paraneoplastic diseases of the retina are rare. Diagnosis can be challenging, and studies on management strategies are limited. The purpose of this review is to summarize our current understanding of the presentation, diagnosis, and management for cancer associated retinopathy, melanoma associated retinopathy, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation.

Multimodal imaging can aid in the diagnosis of paraneoplastic retinopathies since early fundus findings and symptoms can be nonspecific. No standardized treatment protocol exists with therapies having variable efficacy. Generally, systemic immunosuppression is used with possible concomitant local steroid treatment with improved visual outcomes in some cases.

Paraneoplastic syndromes affect the retina as an autoimmune response to tumor antigens or to circulating tumor-derived factors. Diagnosis hinges on maintaining high degree of clinical suspicion, and multimodal imaging can also be helpful. Once diagnosis is made, prompt systemic work-up for malignancy is necessary. Limited literature exists to suggest superiority of a single treatment, but management involves treatment of the underlying malignancy and local or systemic immunosuppression and/or antibody removal.