Primary vitreoretinal lymphoma masquerading as intermediate uveitis in a 30-year-old male

A 30-year-old male presented with floaters and blurred vision in his left eye for 3 months. His visual acuity was 6/6 bilaterally. Fundus examination in the left eye showed dense vitritis obscuring the disc and retina. With a working diagnosis of intermediate uveitis, he was initiated on treatment with oral corticosteroids and mycophenolate mofetil. Due to persistent inflammation, therapy was escalated to the injection of adalimumab. Owing to the lack of remission, the patient underwent left eye diagnostic vitrectomy, which revealed atypical lymphoid cells, and immunohistochemistry confirmed B-cell lymphoma. MYD88 L265P mutation supported the diagnosis of primary vitreoretinal lymphoma (PVRL). Magnetic resonance imaging (MRI) of the brain and Positron emission tomography–computed tomography (PET-CT) were normal. Subsequent vitrectomy in the right eye confirmed bilateral involvement. The patient was referred to oncology and initiated on systemic therapy, including high-dose intravenous methotrexate, intravenous rituximab, and oral temozolomide. Clinicians should maintain a high index of suspicion for masquerade syndromes like PVRL in cases of treatment-refractory intermediate uveitis.