Primary CNS Lymphoma
Overview
Plain-Language Overview
Primary CNS Lymphoma is a rare type of cancer that starts in the brain or spinal cord, which are parts of the central nervous system. It involves the uncontrolled growth of abnormal lymphocytes, a type of white blood cell important for fighting infections. This condition mainly affects the brain's ability to function properly, leading to symptoms like headaches, confusion, weakness, or changes in vision. Because it occurs in the central nervous system, it can cause serious problems with movement, speech, and memory. Diagnosis often requires specialized imaging and tissue sampling to confirm the presence of cancerous cells. Treatment usually involves chemotherapy and sometimes radiation, aiming to control the tumor and improve neurological function.
Clinical Definition
Primary CNS Lymphoma is a highly aggressive non-Hodgkin lymphoma confined to the brain, spinal cord, leptomeninges, or eyes without systemic involvement at diagnosis. It is predominantly a diffuse large B-cell lymphoma arising from malignant transformation of B lymphocytes within the central nervous system. The pathogenesis involves immune dysregulation, often seen in immunocompromised patients such as those with HIV/AIDS or post-transplant immunosuppression, but it can also occur in immunocompetent individuals. Clinically, it presents with focal neurological deficits, neuropsychiatric symptoms, or signs of increased intracranial pressure. The disease is significant due to its rapid progression and poor prognosis without prompt treatment. Diagnosis and management require multidisciplinary care involving neurology, oncology, and pathology.