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General Information About Primary CNS Lymphoma

Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue of the brain and/or spinal cord.

Lymphoma is a disease in which malignant (cancer) cells form in the lymph system. The lymph system is part of the immune system and is made up of the lymph, lymph vessels, lymph nodes, spleen, thymus, tonsils, and bone marrow. Lymphocytes (carried in the lymph) travel in and out of the central nervous system (CNS). It is thought that some of these lymphocytes become malignant and cause lymphoma to form in the CNS. Primary CNS lymphoma can start in the brain, spinal cord, or meninges (the layers that form the outer covering of the brain). Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).

Having a weakened immune system may increase the risk of developing primary CNS lymphoma.

Primary CNS lymphoma may occur in patients who have acquired immunodeficiency syndrome (AIDS) or other disorders of the immune system or who have had a kidney transplant. For more information about lymphoma in patients with AIDS, refer to the PDQ summary on AIDS-Related Lymphoma Treatment.

Tests that examine the eyes, brain, and spinal cord are used to detect (find) and diagnose primary CNS lymphoma.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.


• Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.


• Slit-lamp eye exam: An exam that uses a special microscope with a bright, narrow slit of light to check the outside and inside of the eye.


• Vitrectomy: A surgical procedure to remove some or all of the vitreous humor (the gel-like fluid inside the eyeball). The fluid is removed through tiny incisions and then viewed under a microscope by a pathologist to check for cancer cells.


• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For primary CNS lymphoma, a CT scan is done of the chest, abdomen, and pelvis (the part of the body between the hips).


• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).


• Lumbar puncture: A procedure used to collect cerebrospinal fluid (the fluid in the spaces around the brain and spinal cord) from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap. Laboratory tests to diagnose primary CNS lymphoma may include checking the protein level in the cerebrospinal fluid.


• Stereotactic biopsy: A biopsy procedure that uses a computer and a 3-dimensional (3-D) scanning device to find a tumor site and guide the removal of tissue so it can be viewed under a microscope to check for signs of cancer.


• Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:
  
  - The number of red blood cells and platelets.
  - The number and type of white blood cells.
  - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  - The portion of the blood sample made up of red blood cells.
  


• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on the following:

• The patient's age and general health.


• The level of certain substances in the blood and cerebrospinal fluid (CSF).


• Where the tumor is in the central nervous system.


• Whether the patient has AIDS.

Treatment options depend on the following:

• The stage of the cancer.


• Where the tumor is in the central nervous system.


• The patient's age and general health.


• Whether the cancer has just been diagnosed or has recurred (come back).

Treatment of primary CNS lymphoma works best when the tumor has not spread outside the cerebrum (the largest part of the brain) and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or other diseases that weaken the immune system.

Staging Primary CNS Lymphoma

After primary central nervous system (CNS) lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the brain and spinal cord or to other parts of the body. When primary CNS lymphoma continues to grow, it usually does not spread beyond the central nervous system or the eye. The process used to find out whether cancer has spread is called staging. The following tests and procedures may be used in the staging process:

• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For primary CNS lymphoma, a CT scan is done of the chest, abdomen, and pelvis (the part of the body between the hips).


• Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and the bone marrow samples under a microscope to look for signs of cancer.


• Slit-lamp eye exam: An exam that uses a special microscope with a bright, narrow slit of light to check the outside and inside of the eye.


• Vitrectomy: A surgical procedure to remove some or all of the vitreous humor (the gel-like fluid inside the eyeball). The fluid is removed through tiny incisions and then viewed under a microscope by a pathologist to check for cancer cells.

Recurrent Primary CNS Lymphoma

Recurrent primary central nervous system (CNS) lymphoma is cancer that has recurred (come back) after it has been treated. Primary CNS lymphoma commonly recurs in the brain or the eye.

Treatment Option Overview

There are different types of treatment for patients with primary CNS lymphoma.

Different types of treatment are available for patients with primary central nervous system (CNS) lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Surgery is not used to treat primary CNS lymphoma.

Three standard treatments are used:
Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated.

High-dose radiation therapy to the brain can damage healthy tissue and cause disorders that can affect thinking, learning, problem solving, speech, reading, writing, and memory. Clinical trials have tested the use of chemotherapy alone or before radiation therapy to reduce the damage to healthy brain tissue that occurs with the use of radiation therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer being treated. Primary CNS lymphoma may be treated with intrathecal chemotherapy and/or intraventricular chemotherapy, in which anticancer drugs are placed into the ventricles (fluid-filled cavities) of the brain.

A network of blood vessels and tissue, called the blood-brain barrier, protects the brain from harmful substances. This barrier can also keep anticancer drugs from reaching the brain. In order to treat CNS lymphoma, certain drugs may be used to make openings between cells in the blood-brain barrier. This is called blood-brain barrier disruption. Anticancer drugs infused into the bloodstream may then reach the brain.

Steroid therapy

Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Glucocorticoids are steroid drugs that have an anticancer effect in lymphomas.

Other types of treatment are being tested in clinical trials. These include the following:
High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options for Primary CNS Lymphoma

Primary CNS Lymphoma Not Related to AIDS

Treatment of primary central nervous system (CNS) lymphoma in patients who do not have AIDS may include the following:

• Chemotherapy.


• Chemotherapy followed by radiation therapy.


• Whole brain radiation therapy.


• A clinical trial of high-dose chemotherapy with stem cell transplant.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

Primary CNS Lymphoma Related to AIDS

Treatment of primary central nervous system (CNS) lymphoma in patients who do have AIDS may include the following:

• Steroids with or without radiation therapy.


• Radiation therapy alone.


• Chemotherapy followed by radiation therapy.

Treatment of primary CNS lymphoma is different in patients with AIDS because the treatment side effects may be more severe. (Refer to the PDQ summary on AIDS-Related Lymphoma Treatment for more information).

Recurrent Primary CNS Lymphoma

Treatment of recurrent primary central nervous system (CNS) lymphoma may include the following:

• Chemotherapy or radiation therapy (if not received in earlier treatment).


• A clinical trial of a new drug or treatment schedule.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

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