Primary central nervous system lymphoma

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Primary central nervous system lymphoma
Synonyms	PCNSL, primary CNS lymphoma
Pronounce	N/A
Specialty	N/A
Symptoms	Headache, seizures, focal neurological deficits, cognitive dysfunction
Complications	Increased intracranial pressure, hydrocephalus, vision problems
Onset	Typically in adulthood
Duration	Variable
Types	Diffuse large B-cell lymphoma
Causes	Unknown, associated with immunosuppression
Risks	HIV/AIDS, organ transplantation, immunosuppressive therapy
Diagnosis	MRI, biopsy, CSF analysis
Differential diagnosis	Glioblastoma, metastatic brain tumors, multiple sclerosis
Prevention	N/A
Treatment	Chemotherapy, radiation therapy, corticosteroids
Medication	Methotrexate, rituximab
Prognosis	Variable, depends on response to treatment
Frequency	Rare
Deaths	Varies

Alternate names

Primary lymphoma, CNS; PCNSL; Primary brain lymphoma; Primary CNS lymphoma

Definition

Primary central nervous system lymphoma (primary CNS lymphoma) is a rare form of non-Hodgkin lymphoma in which cancerous cells develop in the lymph tissue of the brain and/or spinal cord. Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).

Micrograph showing a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli. Brain biopsy. HPS stain.

Cause and risk factors

The exact underlying cause of primary CNS lymphoma is poorly understood; however, people with a weakened immune system (such as those with acquired immunodeficiency syndrome) or who have had an organ transplant appear to have an increased risk of developing the condition.

Signs and symptoms

These and other signs and symptoms may be caused by primary CNS lymphoma or by other conditions. Check with your doctor if you have any of the following:

• Nausea and vomiting.
• Seizures.
• Headaches.
• Arm or leg weakness.
• Confusion.
• Double vision.
• Hearing loss.

Diagnosis

The following tests and procedures may be used:

• Physical exam and health 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‚Äôs 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‚Äôs 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.
• Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (enlarged) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is checked. Pictures may be taken over time to keep track of changes in the size of the tumor.

There are several types of eye exams: Ophthalmoscopy: An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light. Slit-lamp biomicroscopy: An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope.

• 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 (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap.
• 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.
• HIV test: A test to measure the level of HIV antibodies in a sample of blood. Antibodies are made by the body when it is invaded by a foreign substance. A high level of HIV antibodies may mean the body has been infected with HIV.

Treatment

There are different types of treatment for patients with primary central nervous system (CNS) lymphoma. Four standard treatments are used:

• Radiation therapy
• Chemotherapy
• Steroid therapy
• Targeted therapy
• New types of treatment are being tested in clinical trials.
• High-dose chemotherapy with stem cell transplant.

NIH genetic and rare disease info

• NIH info on Primary central nervous system lymphoma

Primary central nervous system lymphoma is a rare disease.

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