Sometimes we can suddenly experience physical problems, right? A headache, difficulty speaking, or a feeling of being out of control. Sometimes we don't pay much attention to these, but they can also be symptoms of some serious illness. Today, we are going to talk about a rare but dangerous type of cancer that affects our central nervous system.
What is CNS lymphoma?
Okay, so let's first see what this central nervous system lymphoma, or `(CNS Lymphoma)` is. `CNS` stands for `Central Nervous System`. Simply put, this is a relatively serious type of cancer that develops in our central nervous system , that is, the brain, spinal cord (the nerve cord inside the spinal column), cerebrospinal fluid (the fluid that surrounds this nervous system), and eyes (because the eyes are very close to the brain). This belongs to the type of lymphoma called `(Non-Hodgkin lymphoma)` .
This `CNS` lymphoma starts in our body's white blood cells called lymphocytes . These lymphocytes are part of our lymphatic system. Did you know, this lymphatic system is a very important part of our immune system . It helps our body fight diseases and infections. So, if this lymphoma only develops in our central nervous system and is not found anywhere else in the body, we call it `primary CNS lymphoma` . However, if the lymphoma is also found in other parts of the body, including the central nervous system, it is called `secondary CNS lymphoma` .
Although this cancer can develop in anyone, it is more likely to develop in people with weakened immune systems , such as those with HIV and AIDS , especially men. People over the age of 65 are also at risk. However, it is a very rare cancer. Even in a country like the United States, only about 1,500 new cases are reported each year. So, no matter what your condition is, your medical team will help you find the best treatment to fight CNS lymphoma.
What are the symptoms of CNS lymphoma?
Now let's see what are the symptoms of this `CNS` lymphoma. Actually, the symptoms vary depending on where the tumor is located . For example, if this tumor is in the membranes that cover the brain and spinal cord ( the `meninges` ), it may not show any symptoms. However, if there is a tumor near the eyes, there may be changes in vision. Or, if there is a tumor in the part of the brain that controls movement, there may be a feeling of numbness, changes in balance such as when walking.
These are the common symptoms of CNS lymphoma:
- Nausea and vomiting
- Weakness in the arms, legs, or face
- Weakness on one side of the body (hemiparesis)
- Hearing impairment, deafness
- Difficulty swallowing (dysphagia)
- Symptoms of increased pressure in the brain (severe headache, confusion, disorientation)
- Vision problems (blurred vision, double vision, floaters )
- Changes in mental status (difficulty speaking, memory loss, feeling sluggish)
- Seizures (this may gradually increase over several days or weeks)
- Inability to control urine and stool (urinary and fecal incontinence)
What causes CNS lymphoma?
Next, let's look at what causes CNS lymphoma. Like other types of lymphoma, CNS lymphoma develops when cells in the lymph nodes start to behave abnormally. These cells multiply uncontrollably , crowding out healthy cells. In CNS lymphoma, the cells that start to grow abnormally are lymphocytes, a type of white blood cell called B cells .
Researchers still don't know exactly why a lymphocyte becomes a cancer cell, but they have identified some factors that increase the risk of developing CNS lymphoma.
Risk factors for CNS lymphoma
Certain conditions that weaken the immune system may increase the risk of developing CNS lymphoma. These risk factors include:
- HIV/AIDS (especially if you have an active Epstein-Barr virus infection)
- Wiskott-Aldrich syndrome (WIS)
- Common variable immunodeficiency state (CVID)
- Ataxia -telangiectasia
- Use of immunosuppressant drugs after an organ transplant
How is CNS lymphoma diagnosed?
Your doctor may recommend various tests and procedures to determine if you have CNS lymphoma. This diagnosis is also called cancer staging . This stage of the cancer helps doctors understand how far the cancer has spread and what treatments are most likely to be successful.
You will need to do these tests:
- Physical exam : Your doctor will check the health of your brain, spine, and eyes. He or she will do a neurological exam to check your central nervous system (CNS). They may also do a slit lamp exam to check for a tumor behind the eye.
- Imaging tests : Your doctor may order an MRI, CT scan , or PET scan to see where the cancer is inside your body. CNS lymphoma rarely spreads beyond the central nervous system. However, it can spread rapidly within that system.
- Blood tests : Blood tests can check your cells for signs of cancer. These include a complete blood count , a blood chemistry study, and an HIV test .
- Tissue and fluid tests : A sample of your spinal fluid, bone marrow , or the tumor itself may be taken to check for cancer cells.
What are the treatments for CNS lymphoma?
In fact, there is no one-size-fits-all approach to treating CNS lymphoma. Instead, your healthcare team will recommend a treatment plan that works best for you based on factors such as your age, whether you have HIV/AIDS, and whether the cancer is newly diagnosed or has come back after treatment (recurrent) . Most often, a combination of treatments will be used.
The treatments for CNS lymphoma are:
- Chemotherapy : This involves using drugs to kill cancer cells. High doses of the chemotherapy drug methotrexate (HD-MTX) are often used to treat newly diagnosed CNS lymphoma.
- Radiation therapy : This uses energy beams to kill cancer cells. One type of radiation therapy that can help relieve symptoms is whole-brain radiation . It kills cancer cells throughout the brain.
- Targeted therapy : This uses substances such as proteins and antibodies to attack cancer cells. Rituximab and ibrutinib are targeted therapies that your doctor may recommend.
- Stem cell transplant : In a stem cell transplant, you are given healthy blood cells to replace blood cells that have been damaged by cancer treatments such as chemotherapy and radiation therapy.
- Clinical trials : Your doctor may suggest that you take part in a clinical trial that tests new treatments. Current trials are studying how effective new targeted therapy drugs and new combinations of chemotherapy drugs are in treating CNS lymphoma.
If you are HIV-positive or have AIDS, you should continue your antiretroviral therapy (ART) while you are being treated for CNS lymphoma.
What are the complications and side effects of treatment?
Treatment for CNS lymphoma can cause side effects . Your doctor will explain these to you in advance. For example, whole-brain radiation kills cancer cells in the brain. However, it can also cause serious side effects that can affect how your brain works. All treatment options have risks.
Talk to your doctor about the benefits and risks of your treatment plan. Ask if they recommend palliative care, which can help you manage side effects and the overall impact of cancer on your life.
What can I expect with this situation?
CNS lymphoma is a fast-growing cancer , and it has a high chance of coming back after treatment. However, your chance of recovery, or prognosis , depends on several specific factors. These factors include:
- your age
- Your overall health status
- Your HIV status
- Where the tumor is located
- The results of your blood chemistry tests (these can show how the cancer has affected your internal organs)
Your medical team will work with you to find the treatment plan that gives you the best chance of survival without reducing your quality of life.
What is the recovery rate for CNS lymphoma?
Researchers report cancer survival rates by looking at how many people with a particular cancer are still alive after a specified period of time, usually five years. The five-year survival rate for people with CNS lymphoma is about 30% . That means that three out of every ten people with CNS lymphoma are alive after five years.
However, these statistics are very general . They don't take into account other specific factors that affect a patient's chances of survival. For example, if your immune system is not weakened, or if the lymphoma has not spread beyond the brain, the outcome is usually better. Also, these survival rates don't show how new treatments are affecting your life expectancy.
Your doctor is the best person to explain what your future holds based on your health status and your specific cancer diagnosis.
How do I take care of myself?
Living with lymphoma can be really hard . You may feel anxious about what tests and treatments you will have to undergo. You may not know how to share your feelings with others.
At times like these, you should try to get all the support you can . That might mean talking to your loved ones, even if it's hard. Or it might mean asking your doctor about palliative care or support groups . Everyone's cancer journey is different. But it's important to connect with others at every step of the journey.
When should I see the doctor?
Even if your CNS lymphoma is in remission (that is, you no longer have any signs or symptoms of the cancer), you will still need to have regular check-ups to check for any recurrence. You will need to see your doctor more often during the first five years after treatment. This is because most cases of CNS lymphoma that come back occur during the first five years.
What questions should I ask the doctor?
Here are some questions you can ask your doctor:
- What tests will I need to have to determine my treatment plan?
- What are the risks and benefits of different treatment options?
- How often will I have to come in for tests to see how the cancer is responding to treatment?
- Am I eligible to participate in a clinical trial ? Do you recommend doing so?
- Would you recommend palliative care for me?
The most important message to take home with you
CNS lymphoma is a serious condition that requires close monitoring and a treatment plan tailored specifically to you. It is important to work closely with a medical team that has experience treating lymphoma or cancers that affect the central nervous system (CNS).
Your medical team can explain your treatment options, help you weigh their benefits and potential risks or side effects, and connect you with palliative care specialists who can provide you with comfort as you decide on your next steps.
` CNS lymphoma, central nervous system, cancer, brain cancer, lymphoma, immune system, treatment
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