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Primary Central Nervous System (CNS) Lymphoma: Information on Causes, Diagnosis & Treatments

Nervous System Cancer Large Form Primary Central Nervous System (CNS) Lymphoma is a non-Hodgkin cancer that involves primary intracranial tumors that tend to affect individuals with severe autoimmune deficiency conditions including AIDS. However, it also affects non-AIDS populations. Often referred to as micro-glioma, diffuse histiocytic lymphoma, and reticulum cell sarcoma, primary CNS lymphoma is an immunoblastic or large form of cancer.

The disease begins to develop in the brain, spinal cord, leptomeninges (pia mater and arachnoid), or eyes. The cancer is usually confined to the central nervous system but on rare occasions can spread (metastasize) to other areas of the body. Because of that, medical science classifies the condition is a stage II disease.

The cancer's name is described to be primary to the central nervous system. This means that the cancer initially developed in this region as compared to secondary lymphoma that has metastasized or spread from another area of the body into the central nervous system. The most common forms of primary CNS lymphoma involve:

  • Cancer that has developed in the brain as a mass (lump) as primary CNS lymphoma or metastasized within the meninges as diffuse meningeal lymphoma

  • Cancer that developed in the eye (intraocular lymphoma)

  • Cancer the developed in the spinal cord that produces spinal cord compression

  • Cancer that has initiated in another area of the body resulting in secondary central nervous system lymphoma.

Who Is at Risk for Primary Central Nervous System Lymphoma?

To date, medical science has not determined the exact cause of Primary Central Nervous System Lymphoma. Scientists believe there are specific risk factors for developing the disease including those with a compromised immune system involving chronic amino suppression, immune system disorder, HIV and/or AIDS (acquired immunodeficiency syndrome). Patients who have undergone an organ transplantation also have an elevated risk of developing primary CNS lymphoma.

Recent studies have shown that nearly 6% of all patients suffering from AIDS (acquired immunodeficiency syndrome) will develop primary central nervous system lymphoma while they are ill. Around 20% of all individuals with an HIV infection and less than 90% of patients with Epstein-Barr virus infections develop primary CNS lymphoma. People at greatest risk of developing primary CNS lymphoma tend to be older individuals suffering from the infections listed above when they are in their 50s and 60s.

Common Symptoms

The type of symptoms the patient experiences will be based on the location of the lymphoma. More than 30% of all patients diagnosed with CNS lymphoma have at least one lump of cancerous tissue in the brain. The most common symptoms associated with primary CNS lymphoma include:

  • Balancing problems

  • Vomiting and nausea

  • Headaches

  • Seizures

  • Partial loss or changes in vision

  • Loss of sensation

  • Isolated muscle weakness such as in one extremity

  • Vague confusion

  • Increasing irritability

  • Personality changes

  • Diminished attention involving the inability to remain focused

  • Expressive Dysphasia where the right word is difficult to find when speaking

If the condition involves any portion of the brain, it has the potential of blocking the flow of cerebrospinal fluid that can cause a buildup of cerebrospinal fluid around the brain, leading to intracranial hypertension, excess pressure on the brain. When this condition develops, the patient will experiences a variety of symptoms that include:

  • Vomiting

  • Intense headaches

  • Unresponsiveness or drowsiness caused by a change in consciousness

Any complication associated with Primary Central Nervous System Lymphoma requires immediate medical attention. The chance of recovery (prognosis) for individuals with the condition is based on a variety of factors including the available options for treating the disease. The most common prognostic factors associated with primary CNS lymphoma include:

The overall health and age of the patient

The level of progression

The level of lactate dehydrogenase

If the patient is suffering from AIDS

The tumor location in the CNS

Unusually low levels or high levels of specific substances found in cerebrospinal fluid and blood.

Diagnosing Primary Central Nervous System Lymphoma

To accurately diagnose a case of primary Central nervous system lymphoma, the doctor will perform a comprehensive physical examination an take an accurate personal medical history. Additionally, the doctor will order a variety of tests and procedures that include:

  • Neurological Examination to help evaluate coordination, mental status, reflexes, senses, muscle functioning and the ability to walk without challenges.

  • Imaging – This includes CT (computerized tomography) scans and chest x-rays to gather a visual image of the abdomen and chest areas. They can also include an MRI (magnetic resonance image) of the spinal cord and brain.

  • Slit Lamp Eye Examination – This comprehensive test utilizes a special microscope that has a narrow, bright slip of intense light for better viewing of the inside and outside of the eye.

  • Spinal Tap (Lumbar Puncture)

  • Vitrectomy – This surgical procedure removes a small portion of the eyeball's gel-like fluid (vitreous humor) for an examination under a microscope to detect or identify cancer cells.

  • Stereotactic Biopsy that involves a 3D scanning device and computer to isolate tumors and guide the device to remove affected tissue for an evaluation by a pathologist looking under the microscope to identify tissue samples that contain cancer.


After the doctor verifies the diagnosis of primary central nervous system lymphoma, the cancer must be staged to help determine the best treatment plan. Staging helps identify the progression of the cancerous mass and whether it has spread (metastasized) to other areas of the body. Staging requires many of the same tools used to diagnose the condition that includes:

  • PET scan

  • CT scan

  • Vitrectomy

  • Slit-lamp eye examination

  • Bone marrow biopsy and aspiration

  • Testicular exam in males

Treating the Condition

Medical science has yet to develop a single treatment for primary CNS lymphoma. Usually, the best treatment options are based on the disease, its location, the patient's health, and other factors. Usually, the treatment will be recommended by an oncologist or hematologists who specializes in non-Hodgkin lymphoma. To ensure the most successful outcome, it is best to consider obtaining a second opinion to discuss at length every available treatment option.

Available primary CNS lymphoma treatments for individuals who are not suffering from AIDS include:

  • Chemotherapy

  • Steroid therapy

  • Chemotherapy used concomitantly with radiation therapy

  • Whole brain radiation

  • clinical trials involving stem cell transplantation following high-dose chemotherapy

Available primary CNS lymphoma treatments for individuals suffering from AIDS include:

  • Radiation

  • Steroids

  • Steroids with radiation

  • Radiation therapy that is given after chemotherapy

Treating recurrent primary central nervous system lymphoma involves:

  • Chemotherapy and radiation therapy

  • Chemotherapy (alone)

  • Clinical trials

In many cases, clinical trials offer the best and most successful option for treatment. However, not every individual is an ideal candidate for a clinical trial. Only the doctor handling the case can determine if the patient meets the criteria required for participation.


The prognosis (outlook) after receiving treatment for primary central nervous system lymphoma is best when the condition or tumor is diagnosed before it has spread outside the largest part of the brain (cerebellum). Additionally, individuals 60 years old and younger with primary CNS syndrome in its initial stage have a high success rate of living longer and maintaining daily functions, as long as they do not suffer from a weakened immune system or have AIDS.


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