Abstract

Primary Vitreoretinal Lymphoma (PVRL) is a rare and aggressive type of intraocular lymphoma that affects the eye. PVRL is most commonly diagnosed in individuals in their sixth to eighth decade of life, but it can occur in younger, immunocompromised individuals. PVRL is challenging to diagnose due to its non-specific presentations, and a delay in diagnosis from the onset of symptoms can exceed 12 months. CNS involvement in PVRL is common, and patients may develop CNS involvement within a mean interval of 8-29 months after diagnosis. We present a case of a 73-year-old man who presented to the emergency department with gradual decline in vision in his left eye over the past two years, with acute worsening over the past two weeks. The patient was diagnosed with PVRL with CNS involvement based on exam, imaging and biopsy findings. The patient received intravitreal injection of methotrexate in the left eye and systemic treatment with rituximab and ibrutinib. The patient’s visual acuity improved in the left eye, and regression of tumor size was noted on the fundus exam, OPTOS, and OCTA. Prompt referral to a medical center and early diagnosis are essential in managing PVRL to prevent potential vision loss. Various imaging modalities such as fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography can be helpful in diagnosis and management of PVRL. However, the use of optical coherence tomography angiography has been only described in few case studies. The management of PVRL remains a challenge, with limited treatment options available, including systemic chemotherapy and/or radiation therapy

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