Central Nervous System Lymphoma

Abstract

Primary CNS lymphoma (PCNSL) is a subtype of non-Hodgkin’s lymphoma located in the CNS. Its clinical presentation can be similar to that of acute disseminated encephalomyelitis (ADEM) or tumefactive multiple sclerosis. However, cases of ADEM tend to have a preceding illness and commonly involve systemic symptoms such as fever and weight loss. Imaging shows lesions predominantly in white matter. In contrast, cases of PCNSL present with mono- or multifocal neurological deficits, and imaging shows lesions in supratentorial regions often in contact with ventricular and meningeal surfaces. Diagnosing PCNSL through CSF cytology can be challenging due to the sporadic presence of malignant cells, often requiring multiple lumbar punctures. Thus, brain biopsy is the gold standard for diagnosis, and it is critical to obtain it prior to the administration of steroids that can alter the pathological findings. Chemotherapy is the mainstay of treatment and usually includes methotrexate in combination with cytarabine, with or without radiation. Prognosis of PCNSL can be poor, but overall survival can be increased with treatment, and most patients achieve at least a partial clinical response.