Paraneoplastic syndromes and the retina

Area of Science:

• Ophthalmology
• Oncology
• Immunology

Background:

• Paraneoplastic retinopathies are rare ocular manifestations of cancer.
• Diagnosis can be challenging due to nonspecific early symptoms and fundus findings.

Purpose of the Study:

• To review the presentation, diagnosis, and management of key paraneoplastic retinopathies.
• Includes cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation.

Main Methods:

• Literature review summarizing current understanding.
• Emphasis on multimodal imaging for diagnosis.
• Discussion of treatment strategies.

Main Results:

• Multimodal imaging aids diagnosis of paraneoplastic retinopathies.
• No standardized treatment protocol exists; therapies show variable efficacy.
• Systemic immunosuppression, potentially with local steroids, improves outcomes in some cases.

Conclusions:

• Paraneoplastic retinopathies result from autoimmune responses to tumor antigens or factors.
• High clinical suspicion and multimodal imaging are key for diagnosis.
• Management requires treating the underlying malignancy and employing immunosuppression or antibody removal.