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Primary Central Nervous System (CNS) Lymphoma is a malignant neoplasm of a certain type of white blood cell (cells derived from lymphocytes) that is localized to the Central Nervous System (CNS). Primary CNS Lymphoma can only be diagnosed after the possibility of Lymphoma, originating elsewhere in the body, has been excluded.

These tumors are unusual and represent approximately 1.5% of all primary Brain tumors. It appears that the incidence of these tumors is rising relative to other Brain lesions, in part, due to the occurrence of Primary Lymphoma in HIV-AIDS and Organ Transplant patients. The median age of diagnosis is 52 years. Immunocompromised patients have a younger median age at diagnosis of 34 years. The Frontal Lobes, the deep Thalamic Nuclei and Periventricular zone are the most common supratentorial locations for these tumors.

There are certain conditions that have been associated with an increased risk of Primary CNS Lymphomas. Among these are: Collagen Vascular diseases (Systemic lupus Erythematosus, Sjogren's Syndrome, Rheumatoid Arthritis 2) and diseases which manifest immunosuppression such as Chronic Immunosuppression in organ transplant patients, Severe Congenital Immunodeficiency Syndrome and HIV-AIDS (where CNS lymphoma occurs in 1.9% of AIDS patients) as well as patients with Epstein-Barr virus.


  1. Non-focal, non-specific symptoms occur in at least half of all these patients. These include mental status change (33%) as well as symptoms of increased intracranial pressure (headache, nausea/vomiting).

  2. Generalized seizures occur in 10% of these patients.


MRI scan is the most effective neuroimaging technique for evaluating this condition.

Cerebrospinal Fluid (CSF) examination can be helpful in some cases. The biochemical analysis is augmented with microscopic pathology evaluation.



Surgical management is usually of limited benefit for these patients. Some form of Biopsy technique is the most frequent procedure with Stereotactic methods being the most frequently utilized.

Primary CNS Lymphoma is not amenable to surgical removal by virtue of its pathological uniqueness in not being a "discrete" lesion, meaning that the tumor is not confined to one well circumscribed region.

Combined Therapy

Modern Chemotherapy combined with Radiation Therapy are the primary forms of treatment.


National Cancer Institute


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This page last edited on 2/19

All content ©2022 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.