CNS Lymphoma

What Is CNS Lymphoma?

A central nervous system lymphoma (CNS lymphoma) is a cancer of the lymphatic tissues of the brain, the spinal cord, the meninges or membranes covering the brain (called meningeal lymphoma), and can extend into the eye (known as intraocular lymphoma). It is more commonly found in patients in their 60s or 70s, and sometimes is linked to an immunosuppressed condition.

When the lymphoma originates in the central nervous system it is called primary CNS lymphoma, and if it begins somewhere else and then extends into the CNS system, it is known as a secondary CNS lymphoma.

The majority of CNS lymphomas are of B-cell origins, but the range does include other cells such as T-cells and microglial cells.

What is Brain Lymphoma?

A primary brain lymphoma is rare, arising from lymphatic cells found within the brain. They represent less than 1 percent of all central nervous system (CNS) tumors. A brain lymphoma, or cerebral lymphoma, can occur at any age, but is more common in people ages 45 – 70. Patients who have a weakened immune system are at greater risk.

Brain lymphoma spreads by infiltration of the cerebrospinal fluid and seeding within the central nervous system. In rare cases (fewer than 10 percent), the disease spreads to other organs, bones and soft tissues. Most central nervous system lymphomas are diffuse large B-cell lymphomas.

What Are the Causes and Symptoms of CNS Lymphoma?

Having an impaired immune system can increase your risk of developing CNS lymphoma. Conditions such as immunosuppression of the system after a transplant to reduce organ rejection or acquired immune deficiency syndrome (AIDS) have been linked to higher rates of CNS Lymphoma.

Symptoms of central nervous system lymphoma depend, in part, on where the cancer is located. If the lymphoma is within the brain or meninges, symptoms can include

• Vision changes
• Seizures
• Headache
• Nausea and vomiting
• Coordination issues

Other issues that may present with time:

• Concentration problems
• Confusion, and
• Personality changes

If the cancerous lymphatic cells block the flow of cerebrospinal fluid, the pressure inside the cranium can increase causing more critical symptoms such as sleepiness and a lack of responsiveness, in addition to vomiting and headaches.

Compression on the spinal cord from a tumor can bring about other symptoms:

• Loss of sensation or weakness, usually in the legs,
• Issues with bladder or bowels, and
• Back pain

Intraocular lymphoma, lymphoma that has spread to the eyes, may present symptoms of blurred vision and floaters—shadows and spots that float in the field of vision.

How Is CNS Lymphoma Diagnosed?

Physicians have a number of tools to help make an accurate diagnosis:

• Medical history and a physical examination of general health markers.
• Neurologic exam to gauge the functionality of your central nervous system by assessing your mental function, balance and gait.
• An ophthalmologic evaluation including a slit-lamp eye exam that employs a very bright light to inspect your eye for intraocular lymphoma symptoms.
• Brain imaging tests such as magnetic resonance imaging (MRI), computed tomography (CT) scans and positron emission tomography (PET) scan.
• A lumbar puncture, sometimes called a spinal tap, allows your physician to examine your spinal fluid for unusual cells.
• A stereotactic brain biopsy uses computerized 3-D brain mapping to locate and draw a tissue sample of a mass for an accurate diagnosis.

State-of-the-Art Treatments for CNS Lymphoma at NSPC

Steroids are given to reduce inflammation of the tissues around the lymphoma, especially if there is heightened intracranial pressure due to slowed or arrested cerebrospinal fluid (CSF) flow. Generally, if primary CNS lymphoma is suspected, steroids won’t be introduced until after a stereotactic brain biopsy.

A ventriculoperitoneal shunt may also be recommended to help relieve intracranial pressure from CSF accumulation.

Chemotherapy is the dominant treatment for CNS lymphoma. Intrathecal chemotherapy injects the medication directly into the space in the spinal cord. An Ommaya reservoir may be recommended to avoid needing numerous lumbar punctures to deliver intrathecal chemotherapy.

Treatment Options for Brain Lymphoma

Radiotherapy

Radiotherapy such as gamma knife radiosurgery may be especially advantageous for those with recurrent CNS lymphoma.

Advanced Treatments for Brain Lymphoma at NSPC

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