Paraneoplastic ocular syndromes: a systematic review of epidemiology, diagnosis and outcomes (2010–2023)
Abstract
Paraneoplastic ocular syndromes are rare, immune-mediated disorders triggered by malignancies. They may precede cancer diagnosis or signal its recurrence, highlighting their potential value as early warning signs. Their recognition is critical for timely diagnosis and appropriate management. We performed a systematic review of case reports and case series published between 2010 and 2023 in PubMed database, focusing on six major syndromes: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), bilateral diffuse uveal melanocytic proliferation (BDUMP), acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM), paraneoplastic uveitis (PU), and paraneoplastic optic neuropathy (PON). We extracted demographic, clinical, immunologic, oncologic, therapeutic, and outcome-related data. A total of 132 articles comprising 147 patients were included: 53 with CAR, 22 with MAR, 26 with BDUMP, 16 with AEPPVM, 11 with PU, and 19 with PON. Visual impairment was bilateral in over 90% of cases. The most frequently associated malignancies were lung cancers (notably small-cell lung carcinoma), gynecological cancers, and melanoma. Onconeural autoantibodies were tested in serum—most commonly revealing anti-recoverin and anti-alpha-enolase in CAR, and anti-CRMP5 in PON—but were never assessed in cerebrospinal fluid (CSF), despite its potential diagnostic value. Therapeutic approach was highly heterogeneous and largely empirical, with systemic corticosteroids…
How strong is the evidence?
Evidence Hub reads this as a systematic review, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 147 participants or records.
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