Paraneoplastic Syndromes

Paraneoplastic syndromes are rare but important causes of intraocular inflammation that may mimic primary uveitic conditions. This review highlights the spectrum of paraneoplastic retinopathies, including cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and CRMP5-IgG-associated variants, along with even less common entities such as acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) and bilateral diffuse uveal melanocytic proliferation (BDUMP). While these syndromes may have subtle or misleading clinical presentations, ancillary testing with electroretinography (ERG), visual field assessment, and multimodal imaging can help distinguish them from true uveitides. In addition, rare paraneoplastic uveitic presentations, such as Vogt-Koyanagi-Harada-like disease or sarcoid-like uveitis, reinforce the need for diagnosing clinicians to remain observant of underlying malignancy. Recognizing these masquerading entities is essential to avoid misdiagnosis, guide appropriate treatments, and prompt systemic evaluation for cancer.