Neoplastic Masquerade Syndromes Secondary to Uveal Lymphoid Proliferations
The uveal tract may be a site for benign reactive uveal lymphoid hyperplasia that can mimic chronic uveitis. Presenting symptoms may include vision loss that is gradual, painless, and unilateral or bilateral. Early-stage disease shows multifocal creamy choroidal lesions that may mimic sarcoid uveitis or birdshot uveitis. Macular edema may be present. Anterior uveitis with acute symptoms of pain, redness, and photophobia may also be present. Glaucoma and elevated intraocular pressure (IOP) are common. Angle structures may be infiltrated by lymphocytes, resulting in elevation of IOP. There may be overlap in presentation with posterior scleritis and uveal effusion syndrome.
Fleshy episcleral or conjunctival masses that may appear salmon-pink may be present. Unlike subconjunctival lymphomas, these masses are not mobile and are attached firmly to the sclera. Biopsy specimens demonstrate mature lymphocytes and plasma cells, quite different from the appearance of specimens with PCNSL.
Therapy using corticosteroids, radiation, or both has been used with variable results. Systemic and periocular corticosteroid therapy can lead to rapid regression of the lesions, as can external-beam radiation.
Excerpted from BCSC 2020-2021 series: Section 9 - Uveitis and Ocular Inflammation. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.