PRESENTING AUTHOR

To report four different clinical presentations of vitreoretinal lymphoma (VRL) and therapeutic management.

Case 1: A 60-year-old woman referred painless decreased vision and floaters in right eye. Diagnosis: primary VRL. Initial treatment: intravitreal Rituximab. Subsequently, she developed left eye and central nervous system (SNC) involvement. Treatment: bilateral intravitreal rituximab plus systemic chemotherapy. Case 2: A 57-year-old woman, debuted with painless decreased vision and floaters. She was misdiagnosed as bilateral uveitis. Diagnosis: bilateral VRL. Treatment: systemic and intravitreal Rituximab, then switched to intravitreal Methotrexate. Subsequent CNS involvement. Case 3: A 70-year-old man, referred with diagnosis of bilateral uveitis. Medical history: testicular lymphoma (diffuse large B-cell non-Hodgkin lymphoma). Diagnosis: secondary bilateral VRL. Treatment: intravitreal Rituximab. Case 4: A 59-year-old woman, misdiagnosed as having posterior vitreous detachment. Fundus exam: vitritis. Brain MRI: lesions with inflammatory appearance. Brain biopsy: high-grade aggressive B lymphoma. Vitrectomy and biopsy: type B lymphoproliferative syndrome. Treatment: intravenous rituximab - MATRIX (systemic), intravitreal rituximab, and allogeneic bone marrow transplant.

VRL is a rare malignancy characterized by poor long-term prognosis. Due to its rarity, diagnosis and treatment represents a challenge. It often mimics chronic uveitis in middle to advanced ages that partially responds to corticosteroids. Available therapies include systemic chemotherapy, local treatments, or a combination of both.

Clinical suspicion is essential to accomplish diagnosis in VRL. Ruling out CNS involvement is mandatory. A multidisciplinary approach with an hematologist is crucial. Early recognition with prompt and aggressive treatment is essential to improve prognosis of VRL.