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Lymphoma of the Central Nervous System

(also called "primary CNS lymphoma" or "PCNSL")


 

Definition: Lymphoma is a cancer that arises from cells of the immune system. Primary central nervous system lymphoma (PCNSL) arises from immune system cells in the brain or spinal cord and can spread throughout the central nervous system.  It can also be found in the eyes and other parts of the body.

Symptoms: The most common symptoms of CNS lymphoma are changes in behavior, weakness on one side of the body, seizures, or symptoms of increased intracranial pressure such as headache, nausea, vomiting, and vision changes.

Evaluation: An MRI or CT scan can confirm the presence of CNS lymphoma. If high-dose steroids are taken, the tumor may seem to shrink on a brain scan. A lumbar puncture (spinal tap) may be performed to determine whether there are lymphoma cells in the cerebrospinal fluid. An examination with an ophthalmologist is usually recommended to assess for the presence of tumor in the eyes.

Treatment Options:

  • Surgery: Surgical biopsy is necessary to confirm the diagnosis of CNS lymphoma. Maximal surgical removal does not improve the outcome for people with CNS lymphoma like it does for people with most other types of brain tumors.  Removal of a portion of the tumor is recommended in some cases to relieve pressure on the brain.
  • Radiation:  Radiation therapy may be a treatment considered for CNS lymphoma.
  • Chemotherapy:   Chemotherapy is the primary method of treatment for CNS lymphoma. Importantly, the chemotherapy used differs significantly from other brain tumors, making accurate diagnosis critical in these cases.
  • Follow-up: Patients with primary CNS lymphoma are followed at regular intervals with MRIs.