Primary Vitreoretinal Lymphoma Masquerading as Acute Retinal Necrosis Syndrome, Neovascular Glaucoma, and White Dot Syndromes: A Case Report

Abstract Background: Primary vitreoretinal lymphoma (PVRL) is a high-grade malignancy of non-Hodgkin lymphoma, with a wide range of clinical manifestations. In this report, we described a case of PVRL initially masquerading as acute retinal necrosis and neovascular glaucoma in the right eye and white dot syndromes in the left eye. The disease was finally diagnosed as diffuse large B-cell non-Hodgkin lymphoma. In addition, we reviewed the current diagnostic methods, multimodal imaging findings, and treatment of PVRL. Case presentation: A 50-year-old woman presented with blurred vision and redness in her right eye. Funduscopic examination showed severe vitreous opacification with faintly visible hemorrhages and exudates on the retinal surface. As the disease progressed, rubeosis and neovascular glaucoma developed in the right eye. At the same time, fundus examination of the left eye demonstrated multiple yellow-white spots, which are similar to “white dot syndromes”. She was eventually pathologically diagnosed with intraocular lymphoma after right eye enucleation. The left eye stabilized with continuous intravitreal methotrexate injections. Conclusions: The clinical manifestation of PVRL is often atypical. Multimodal imaging, particularly fundus photography and optical coherence tomography, facilitates appropriate clinical suspicion. Negative cytological testing of the aqueous humor in the anterior chamber may not be sufficient to rule out PVRL. Pathology remains the gold standard for the diagnosis of PVRL.