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.
- 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).
- 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.
Modern Chemotherapy combined with Radiation Therapy are
the primary forms of treatment.
National Cancer Institute
lymphomation.org
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This page last edited on 2/19
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