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Primary Central Nervous System Lymphoma (PCNSL)

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Posted by fatima.shakeel@opmc.co | Feb 7, 2025

What is Primary Central Nervous System Lymphoma?

Primary Central Nervous System Lymphoma (PCNSL) is a rare malignancy that develops in the brain, spinal cord, or eye. It is formed from lymphocytes, which are white blood cells that help fight infections. This form of cancer does not spread to other areas of the body.

Disease Causes

Although the exact cause of Primary Central Nervous System Lymphoma is unknown, it is more common in patients with compromised immune systems, such as those who have received organ transplants, HIV/AIDS, or inherited immunological diseases. Some cases may also involve the Epstein-Barr virus (EBV), which causes mononucleosis.

Signs and Symptoms

Symptoms vary according to the location of the tumour and may occur gradually. The following are typical symptoms: 

  • Arm or leg weakness; 
  • Memory loss or confusion
  • Personality or behavioural changes
  •  Blurred or lost eyesight
  • Headaches and nausea (from swelling in the brain)
  • Seizures (although less often than in other brain tumours)
  •  Trouble walking or maintaining balance of the body

Diagnosis

A multistep diagnosis is needed to diagnose PCNSL.

  • Imaging Tests: A CT-Scan or MRI can be used to identify the location, size, and blood supply of the tumour.
  • Biopsy: A small tissue sample is taken from the tumour for analysis under a microscope.
  • Immunohistochemistry: It is a special test to rule out other types of tumours.
  • Lumber Puncture (spinal tap) to detect cancer cells in the spinal fluid
  • Complete blood count: To assess the function of immune system and to detect any infection marker
  • Eye Exam to detect the extent or origination of the tumour cells in the eyes

Treatment 

Chemotherapy is the primary treatment, frequently combined with high-dose methotrexate (HD-MTX) to assist reduce the tumour’s size. Additional therapies could consist of: 

  • Steroids Medicine:  (to lessen swelling in the brain) 
  • Radiation therapy (sometimes used, but may impair memory) 
  • Stem cell transplantation (for individuals who experience a recurrence following chemotherapy) 
  • Targeted therapy, which involves testing novel medications in clinical studies. 
  • HIV therapy (if AIDS is present) 

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