Primary oculocerebral lymphoma

Primary oculocerebral lymphoma, also known as primary central nervous system lymphoma with ocular involvement or primary intraocular-central nervous system lymphoma, is a rare and aggressive form of non-Hodgkin lymphoma that primarily affects the brain, spinal cord, leptomeninges, and eyes. It is classified as a diffuse large B-cell lymphoma in the vast majority of cases. The disease can present initially in the eye (vitreoretinal lymphoma) and subsequently involve the brain, or it may affect both sites simultaneously. This condition is distinct from systemic lymphoma that secondarily spreads to the central nervous system. Key symptoms depend on the site of involvement. Ocular manifestations include blurred vision, floaters, decreased visual acuity, and painless vision loss, often mimicking chronic uveitis. Cerebral involvement may cause cognitive decline, personality changes, focal neurological deficits, headaches, seizures, and signs of increased intracranial pressure. The disease can affect one or both eyes and may progress from isolated ocular disease to cerebral involvement over months to years. Primary oculocerebral lymphoma occurs predominantly in adults, with a median age of onset typically in the fifth to seventh decades of life. Immunosuppressed individuals, including those with HIV/AIDS or organ transplant recipients, are at increased risk, though the disease also occurs in immunocompetent patients. Diagnosis requires a combination of ophthalmologic examination, neuroimaging (MRI), cerebrospinal fluid analysis, and tissue biopsy. Treatment typically involves high-dose methotrexate-based chemotherapy regimens, which can cross the blood-brain barrier, sometimes combined with whole-brain radiotherapy or intravitreal chemotherapy (such as methotrexate or rituximab) for ocular disease. Despite treatment advances, prognosis remains guarded with a significant risk of relapse, and overall survival has improved but remains limited compared to systemic lymphomas.

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