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Primary CNS Lymphoma - Symptoms, Diagnosis, and Treatment

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Primary central nervous system lymphoma is caused due to cancer cells in the lymph tissue of the brain and spinal cord. Read the article to know more.

Medically reviewed by

Dr. Rajesh Gulati

Published At September 7, 2023
Reviewed AtDecember 1, 2023

Introduction

Primary CNS lymphoma is a cancer of the brain and spinal cord lymph tissue. A compromised immune system may enhance the chances of acquiring primary CNS lymphoma. Primary CNS lymphoma symptoms may include nausea, vomiting, seizures, and seizures. In addition, certain factors influence recovery and treatment options.

What Is Primary CNS Lymphoma?

The lymph system is a component of the immune system that includes the lymph, lymph veins, tonsils, lymph nodes, thymus, spleen, and bone marrow. Lymphoma is a condition that causes cancerous cells to grow in the lymphatic system. Lymphocytes are cells transported in the lymph and are considered to turn malignant and induce lymphoma.

Primary CNS lymphoma can develop in the brain, spine, or the layers that form the brain's outer covering. Primary CNS lymphoma can begin in the eye near the brain and is called ocular lymphoma. A compromised immune system may enhance the chances of acquiring primary CNS lymphoma. For example, patients with acquired immunodeficiency syndrome (AIDS) or other immune system illnesses are at risk of developing primary CNS lymphoma.

What Are the Signs and Symptoms of Primary CNS Lymphoma?

The signs and symptoms of primary CNS lymphoma are as follows:

  • Vomiting and nausea.

  • Seizures.

  • Headaches.

  • Weakness in arms or legs.

  • Confusion.

  • Doubtful vision.

  • Hearing impairment.

How Is Primary CNS Lymphoma Diagnosed?

The following tests and procedures are performed to diagnose primary CNS lymphoma:

1. Physical Exam and Health History: An examination of the body to examine general health indicators, including lumps.

2. Neurological Exam: A neurological exam is a set of questions and tests designed to assess the brain, spinal cord, and nerve function. The test assesses a person's mental state, coordination, ability to walk normally, and the efficiency of their muscles, senses, and reflexes.

3. Eye Examination: An eye exam dilates the pupil with medicated eye drops to visualize the retina, lens, and pupil. The eye's interior is examined, including the retina and optic nerve. Photographs are taken throughout time to document changes in the tumor size. There are many kinds of eye exams:

  • Ophthalmoscopy: This method involves, examining the back of the eye with a small magnifying lens and a light to evaluate the retina and optic nerve.

  • Slit-Lamp Biomicroscopy: This method involves, examining the inside of the eye with a powerful light and a microscope to evaluate the retina, optic nerve, and other eye elements.

4. MRI (Magnetic Resonance Imaging): It is a process that creates a sequence of detailed images of locations inside the brain and spinal cord using radio waves, magnets, and a computer. A chemical known as gadolinium is injected into the person via a vein. Gadolinium accumulates around cancer cells, making them seem brighter in the image.

5. Lumbar Puncture: Lumbar puncture is done by collecting cerebrospinal fluid (CSF) from the spinal column by inserting a needle between the spine and extracting the liquid. The CSF sample is examined under a microscope for tumor cells. The doctor can also determine protein and glucose levels. A higher-than-average level of protein or a lower-than-usual level of glucose may indicate the presence of a tumor. This operation is known as a spinal tap.

6. Stereotactic Biopsy: A biopsy technique that uses a computer and 3-dimensional (3-D) scanning equipment to locate a tumor and direct the removal of tissue to check for signs of malignancy.

7. Flow Cytometry: This is a laboratory test that determines the number of cells in a sample, the proportion of living cells, and specific cell properties. Cells from a person’s blood, bone marrow, or other tissue are dyed with a fluorescent dye, deposited in a fluid, and then passed through a filter. This test is used to aid in diagnosing and treating some malignancies, such as leukemia (blood cancer) and lymphoma (cancer growth in the lymphatic system).

8. Immunohistochemistry: It is a laboratory test that employs antibodies to look for specific antigens in a person’s tissue sample. Typically, antibodies are coupled to an enzyme or a luminous dye. The enzyme or dye is released once the antibodies attach to a specific antigen in the tissue sample. This test is used to help detect cancer and distinguish one form of cancer from another.

9. Cytogenetic Analysis: This is a laboratory test that counts and examines the cell chromosomes in the blood sample of bone marrow for any alterations, such as damaged, missing, altered, or additional chromosomes. Changes in specific chromosomes may be an indication of malignancy. Cytogenetic analysis is used to aid in cancer diagnosis, plan treatment, and assess how well the treatment works.

10. FISH (Fluorescence In Situ Hybridization): It is a technique for examining and counting genes or chromosomes in cells and tissues. In the laboratory, fluorescent dye-containing DNA (deoxyribonucleic acid) fragments are created and added to a tissue sample. When these colored fragments of DNA bind to specific genes or chromosomal regions in the selection, they light up when examined under a fluorescent microscope. The FISH test is used to aid in cancer diagnosis and therapy planning.

11. Total Blood Count: A technique in which the blood sample is collected and tested for the following:

  • The number of red blood cells and platelets.

  • The quantity and variety of white blood cells.

  • The amount of hemoglobin.

  • The percentage of red blood cells in a blood sample.

12. HIV (Human Immunodeficiency Virus) Test: A test determining the amount of HIV antibodies in a blood sample. A high level of HIV antibodies may indicate that the body is infected with HIV.

What Are the Risks For Primary CNS Lymphoma?

The following factors determine treatment choices:

  • The tumor's location is in the central nervous system, the eye.

  • Age and overall health of the person.

  • Whether the cancer was just discovered or has recurred.

How Is Primary CNS Lymphoma Treated?

Treatment for primary CNS lymphoma is most effective when the tumor has not progressed outside the central portion of the brain, the person is less than 60 years old, capable of doing most everyday activities, and does not have AIDS (acquired immunodeficiency syndrome) or other immune-suppressive disorders. The different treatment options are as follows:

1. Radiation Treatment- Radiation therapy employs high-energy X-rays or other radiation forms to kill or prevent cancer cells from developing. For example, as primary CNS lymphoma spreads throughout the brain, the entire brain is treated with external radiation. This method is referred to as whole-brain radiation treatment.

High-dose radiation therapy to the brain can harm healthy tissue and cause cognitive, learning, problem-solving, reading, writing, speech, and memory impairments. Clinical trials have investigated the use of chemotherapy alone or in conjunction with radiation treatment to minimize the damage to healthy brain tissue caused by radiation therapy.

2. Chemotherapy- It employs medications to halt the proliferation of cancer cells by killing or preventing them from growing. Chemotherapy medications enter the circulation and can reach cancer cells throughout the body. However, when chemotherapy is administered directly into the cerebrospinal fluid, an organ, or a bodily cavity such as the abdomen, the chemicals primarily attack cancer cells in those places (regional chemotherapy).

The chemotherapy is administered differently depending on whether the tumor is in the CNS or the eye. The doctor can treat primary CNS lymphoma with systemic chemotherapy, intrathecal chemotherapy, and intraventricular chemotherapy, which involves injecting anticancer medications into the brain's ventricles. Anticancer medications are administered intravenously when primary CNS lymphoma is found in the eye.

3. Steroid Treatment- Steroids are hormones that the body produces naturally. They can also be created in a laboratory and utilized as medications. For example, glucocorticoids are steroid medications that have anticancer properties in lymphomas.

4. Targeted Therapy- It is a cancer treatment in which medicines or other substances are used to locate and destroy specific cancer cells.

5. Monoclonal Antibodies - These are immune system proteins created in the laboratory to treat various disorders, including cancer. As a cancer therapy, these antibodies can bind to a target-specific to the cancer cells or other cells, which may aid in the growth of cancer cells. The antibodies can then attack the cancer cells, stop their development, or prevent their spread. For example, monoclonal antibodies such as Rituximab and Nivolumab treat newly diagnosed or recurrent primary CNS lymphoma.

6. Tyrosine Kinase Inhibitors - These small-molecule medications enter cancer cells through the cell membrane and disrupt their growth. Ibrutinib is a tyrosine kinase inhibitor used to treat new or recurrent primary CNS lymphoma.

Which Factors Influence the Prognosis of the Therapy?

The following factors influence the likelihood of recovery and treatment options:

  • If the person has AIDS.

  • Age and overall health.

  • The tumor's location is in the central nervous system and the eye.

  • The concentration of certain chemicals in the blood and cerebrospinal fluid (CSF).

Conclusion

Primary CNS lymphoma is an aggressive lymphoma involving the CNS, which includes the brain, spine, cerebrospinal fluid (CSF), and eyes. Unlike other brain cancers, it frequently responds favorably to chemotherapy and radiation treatment, but survival is generally lower when compared to lymphomas outside the CNS. However, with appropriate treatment, the living condition of the patient can be improved.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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