Primary Central Nervous System Lymphoma (PCNSL)

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Synonyms

Microglioma , Primary brain lymphoma,

Overview

Primary Central Nervous System Lymphoma (PCNSL) is a rare, aggressive type of lymphoma that originates in the brain and/or spinal cord, and does not spread to other parts of the body. It’s a subtype of non-Hodgkin lymphoma, often diagnosed in older adults, and is more common in men than women. While PCNSL can occur in anyone, it’s more likely to develop in people with weakened immune systems, such as those with HIV/AIDS or those taking immunosuppressant drugs.

Symptoms

  • Neurological deficits: Weakness, numbness, difficulty with speech, coordination problems.
  • Mental status changes: Confusion, memory loss, behavioral changes.
  • Increased intracranial pressure: Headaches, nausea, vomiting, seizures.
  • Vision changes: Blurred vision, double vision, or vision loss.

Causes

Like other types of lymphoma, CNS lymphoma forms when cells in lymph tissue start to behave abnormally. They multiply out of control and overtake healthy cells. With CNS lymphoma, the cells that start growing abnormally are usually white blood cells (lymphocytes) called B cells.

Researchers aren’t sure what causes a lymphocyte to transform into a cancer cell. But they’ve identified factors that may increase your risk of CNS lymphoma.

Diagnosis

Diagnosis can be challenging due to the variety of symptoms and the need for specialized tests like biopsies, MRI, and PET scans.

Prognosis

The prognosis varies depending on individual factors like age, overall health, and HIV status, but it can be difficult to predict and is often guarded. 

  • Survival rates: Vary depending on factors like age, overall health, and treatment response, but overall survival rates are lower than for other lymphomas. 
  • Relapse: Recurrence is common, even after successful treatment. 
  • Palliative care: Can help manage symptoms and improve quality of life for patients with PCNSL. 

Treatment

Treatment typically involves a combination of steroids, chemotherapy, and sometimes radiation therapy.