What is Choroid Plexus Carcinoma? Let's talk about this dangerous but rare brain cancer!

Sometimes, when we think about the diseases that our loved ones, especially our little ones, suffer, we feel a great burden and fear, don't we? Today we are going to talk about a somewhat serious, but very rare type of brain cancer . Don't be afraid immediately when you hear its name. Because, being aware of any disease is a great strength to face it. So, let's talk in detail about this ``Choroid Plexus Carcinoma``.

What is Choroid Plexus Carcinoma?

Simply put, this `Choroid Plexus Carcinoma` is a cancer that develops in a special tissue called ``choroid plexus`` inside our brain. Just think, there are different parts and different types of tissue inside our brain. This ``choroid plexus`` tissue is located in the cavities (``ventricles``) inside the brain. Like a water balloon, it is this ``choroid plexus`` tissue that mainly helps to make ``cerebrospinal fluid`` (``CSF``) that protects and nourishes our brain and the spinal cord that runs down the spine.

This is a malignant tumor . This means that the cells in it grow out of control, very quickly, and can spread to other places. Doctors also call this a ``grade 3 choroid plexus tumor.'' These grow very quickly and try to spread throughout the ``central nervous system'' (``CNS`') through the ``CSF'' fluid. That's why treating them can sometimes be a bit challenging.

How common is this disease?

You may be surprised to hear this, but this disease called `choroid plexus carcinoma` is actually a very rare disease . Just think, out of all 100 types of brain tumors, less than 1% are `choroid plexus tumors`. There are three types of `choroid plexus` tumors. Among them, this `choroid plexus carcinoma` is the rarest and only type of cancer, that is, the `malignant` type . Sadly, this condition mostly affects babies and young children .

What are the symptoms of this disease? How do we recognize it?

So, what are the symptoms of this condition? This can vary depending on the size of the tumor, its location in the brain, and the age of the child. But, in general, there are a few signs to look out for:

• Constant feeling of extreme tiredness and lethargy: It's as if the child is not alive, they don't run around and play like they used to. They seem to be constantly asleep.
• Headaches: A young child may not be able to articulate this, but we can tell by things like them holding their heads, crying frequently, and refusing to eat.
• Irritability or fussiness: A young child may be fussy and difficult to comfort. They may be more stubborn than before.
• Nausea and vomiting: This may be worse, especially in the morning.
• Numbness or weakness in some parts of the body: An arm or leg may feel as if it is completely numb.
• Seizures: Sudden convulsions and loss of consciousness.
• Vision problems: You may see blurred vision or see two images at once (diplopia). A young child may rub their eyes frequently or have difficulty focusing.
• Enlarged head size: In young babies, the head may enlarge as pressure inside the brain increases because the bones of the skull are not fully fused.

Don't be afraid to have this disease just because you have one or two of these symptoms. However, if these symptoms persist, it is wise to definitely see a doctor .

Why does this kind of disease occur? What is the cause?

In fact, it is difficult for doctors to say exactly why some people develop choroid plexus carcinoma. Usually, any cancer develops when there is a change in one of the genes in our body, which we call a mutation. This genetic mutation causes healthy cells to become cancer cells and multiply uncontrollably, grow into a tumor, and sometimes spread throughout the body.

Are there any risk factors that affect this?

Anyone can develop this condition called `choroid plexus carcinoma`. However, it has been found that certain things (we call them `risk factors`) can increase the chances of developing it. Let's see what they are:

• Aicardi syndrome: This is a very rare genetic condition. Children born with this condition have certain abnormalities in brain development, making them more likely to develop choroid plexus tumors. This is not usually inherited.
• Li-Fraumeni syndrome: This is also a rare genetic condition. People with this syndrome are more likely to develop various types of cancer. This is often caused by a gene change that is passed down from one parent to the child.
• Biological sex: Although the exact reason is not clear, statistics show that women are slightly more likely to develop this disease.

How do doctors diagnose this disease? (Diagnosis)

So, how do doctors confirm whether you have choroid plexus carcinoma? They do this by listening carefully to your symptoms, doing a physical exam, and then doing various tests. These tests may vary depending on your age, or the age of your child:

• Cognitive test: This tests how well you are functioning, such as memory, attention, and learning ability.
• A complete neurological exam: This will check for vision, hearing, balance issues, reflexes, and coordination of different parts of the body.
• An MRI (Magnetic Resonance Imaging) scan of the brain or other imaging tests (such as a CT scan): This is often the most important test. It can show if there are any tumors in the brain, how large they are, where they are located, and whether there is any condition called hydrocephalus (a buildup of cerebrospinal fluid (CSF) in the brain cavities, causing increased pressure).
• DNA test (genetic test): This test can be done to see if there are any genetic changes associated with conditions such as the previously mentioned Li-Fraumeni syndrome or Aicardi syndrome.
• Spinal tap (lumbar puncture): This involves inserting a thin needle into the lower back and taking a small sample of CSF. It is then sent to a laboratory to be tested for cancer cells.

What are the treatments for this?

If it is confirmed that you have a tumor like this, what do you do next? There are several treatment options. The main one is surgery .

• Surgery: Neurosurgeons perform brain surgery to remove the tumor as completely as possible. This is a very complex surgery. A small piece of the removed tumor (a biopsy) is taken and sent to a laboratory to confirm whether it is choroid plexus carcinoma or another type.

If this biopsy confirms choroid plexus carcinoma, and the tumor cannot be completely removed, or if the cancer has spread, you or your child may need additional treatments. These include:

• Chemotherapy: This involves giving the body various types of drugs to kill cancer cells. These drugs kill rapidly dividing cancer cells.
• Radiation therapy: This uses high-energy rays (e.g. X-rays) to target and destroy cancer cells.

One or more of these treatments may be used together. This is decided by a team of specialist doctors, taking into account many factors such as the patient's condition and the nature of the tumor.

What are the possible complications after surgery?

As with any surgery, there is a small chance of complications after brain surgery. Although not common, some of the things that can sometimes be seen include:

• Bleeding or swelling in the brain.
• Difficulty thinking, confusion or delirium.
• Persistent headache.
• Infection at the surgical site.
• Memory loss or difficulty speaking.

Doctors will inform you about these things in advance.

Are new treatments for this disease being developed?

Yes! Medical science is advancing day by day. So, research is continuing to find new, more effective treatments for this disease, ``choroid plexus carcinoma.`` Some of these may still be in the research stage (``clinical trial``). Here are a few examples:

• Immunotherapy: This is a very interesting method. It involves stimulating our own immune system (the system that protects us from diseases) and giving it drugs that help it find and destroy cancer cells.
• Targeted therapy: This involves giving drugs that target specific genetic changes that cause cancer and affect only those changes. This results in less damage to healthy cells.

You can learn more about these new treatments by talking to your doctor.

What is recovery like after treatment?

The time it takes to recover after treatment, including when you can return to work or school, depends on several factors. Here are some of them:

• Your age and overall health.
• The location of the tumor, its size, and how many tumors there are.
• If you received additional treatments such as chemotherapy or radiation therapy, their effects.

Therefore, it is important to follow your doctor's instructions exactly . To monitor whether the healing process is going well, doctors will often ask you to have brain `imaging` tests (e.g. `MRI scans`). Doing everything correctly is very important for your speedy recovery.

What is the prognosis with this disease?

This question is the biggest burden on many people's minds. The outlook for choroid plexus carcinoma, that is, the chance of recovery and life expectancy, varies greatly from person to person. There are several key factors that affect it:

• The patient's age (results may vary for young children).
• The patient's overall health status.
• Whether the entire tumor was removed during surgery, or only part of it. (If the tumor can be completely removed, the outlook is relatively good.)

Also, these things also affect the outlook:

• If the tumor comes back after surgery or other treatments (`recurrence`).
• If the cancer spreads (metastasizes) from its original site to other parts of the body, for example, the spinal cord.

Not everyone is the same, so it's best and most accurate to ask your medical team what to expect based on your condition.

Statistically, the overall five-year survival rate for choroid plexus carcinoma is between 40% and 60%. This means that out of 10 people diagnosed with the disease, 4 to 6 will live for five years or more. However, for someone with the previously mentioned genetic condition Li-Fraumeni syndrome, this percentage is said to be as low as 30 %.

Don't be alarmed by these statistics. These are just averages. Everyone's condition, response to treatment, and battle is different. With the advancement of medical science, these conditions are getting better day by day.

When should we definitely see a doctor?

If you or your child continues to have one or more of the symptoms mentioned above, especially if you see any of the following, you should definitely see a qualified doctor without wasting any time :

• Obvious changes in behavior. For example, sudden confusion, extreme irritability, agitation.
• Difficulty breathing.
• Difficulty walking, loss of balance.
• Sudden seizures (`Seizures`).
• Very severe, unbearable headache.
• Sudden changes in vision or speech.

What are the important questions to ask the doctor?

When you see your doctor, you can ask questions like these to gain a clearer understanding of your condition. Don't be afraid to ask everything that's on your mind:

• "Doctor, exactly what type of brain tumor do I/my child have?"
• "What are the best treatments for this? Which of them is most suitable for me/my child?"
• "What are the possible side effects after treatment?"
• "How often will I/my child need to have tests like `imaging` scans?"
• "What is my/my child's outlook with this condition?"

A word to the family, especially to the parents...

When your child is diagnosed with choroid plexus carcinoma, it's normal to feel like your whole world has come crashing down. Everything starts to happen very quickly. You'll meet a large team of doctors, including new neurologists and oncologists. Your calendar will be filled with tests, doctor's appointments, and surgeries. After the initial excitement, you'll have to wait a long time for the results to come back, for the next steps. This waiting period can be very stressful and difficult.

But, every little moment you can spend with your child during that time is precious. Learn a new lullaby to lull him to sleep, watch his favorite cartoons or movies together, try to find something new that you can do together that brings you joy.

It's hard to live in the present moment, worrying about the next scan, the next test report. But no matter what that scan or report reveals, you can always put aside your worries and fears and be happy for the precious time you spent with your baby. You are not alone. There are many people who can help you and listen to you.

Finally, the most important things to remember (Take-Home Message)

Okay, so let's summarize the most important things you need to keep in mind about this long-discussed ``Choroid Plexus Carcinoma''?

• Choroid Plexus Carcinoma is a rare but rapidly growing type of cancer that develops in the tissue of the brain called the choroid plexus. It especially affects young children and infants .
• If you have one or more symptoms such as excessive sleepiness, persistent headaches, frequent vomiting, seizures, or vision problems due to undiagnosed causes, do not waste time and seek medical advice immediately .
• The main treatments used are surgery, chemotherapy, and radiation therapy . Sometimes more than one of these treatments may be used.
• Although this is a challenging and difficult journey, if you or someone in your family is facing this situation, remember that you are not alone . Get through this with the support of your medical team, family, and friends. Staying positive and strong is very important in this fight.

We hope this information is useful to you. Good health to all!

` Choroid Plexus Carcinoma, brain cancer, childhood cancer, cerebrospinal fluid, cancer symptoms, cancer treatment, brain tumor, childhood cancer