Histopathologic Diagnosis of Atypical Primary Vitreoretinal Lymphoma following Enucleation

Introduction: Primary vitreoretinal lymphoma (PVRL) is a rare but potentially fatal malignancy. The diagnosis of PVRL remains challenging. Here, we present a case of PVRL with a diagnostic enucleation required to establish the diagnosis. Methods/Case Description: A 58-year-old otherwise healthy woman was referred for evaluation of panuveitis with persistent blurred vision in the left eye. Extensive laboratory work-up was negative. On initial evaluation, the patient had visual acuity of no light perception in the left eye. Examination of the left eye revealed diffuse neovascularization of the iris, moderate vitreous debris and no view of the posterior pole. A B-scan ultrasound showed a retinal detachment and extensive subretinal fluid. Enucleation was performed. Results: On gross examination, a white mass was noted under the RPE. Histologic examination showed a mass of large CD20+ lymphocytes under the RPE. Discussion: The histopathologic findings confirmed a diagnosis of large B-cell lymphoma (PVRL). Conclusion: PVRL may masquerade as uveitis, thus leading to a delay in diagnosis and management. A diagnostic enucleation may be useful in blind painful eyes of unknown etiology.