Does your little one have this rare cancer? Let's talk about Rhabdoid Tumor!

Moms and dads, it's a big burden on your heart and something you don't even want to think about when your little one finds out that they have cancer. But sometimes, children can develop illnesses that we don't expect. Today we're going to talk about a very rare, fast-spreading type of cancer that affects children, especially young children. This is called Rhabdoid Tumor . You may feel a little strange when you hear this name, but let's explain it simply.

What is a Rhabdoid Tumor? Simply put...

Rhabdoid Tumor is a rare, fast-growing type of cancer . It is most common in young children and infants . When viewed under a microscope, these cancer cells look similar to rhabdomyoblasts , a type of muscle-forming cell. That's why it's called "rhabdoid." This cancer can develop in a child's kidneys, soft tissues, or central nervous system (brain and spinal cord). Unfortunately, this cancer can spread very quickly, making it difficult to treat.

Are there different types of Rhabdoid Tumor?

Yes, there are several main types of this cancer depending on where it occurs. Let's see what they are.

1. Rhabdoid Tumor of the Kidney (RTK)

This is rhabdoid tumor of the kidney in a child. It is sometimes abbreviated as ``(RTK)``.

2. Extracranial or Extrarenal Malignant Rhabdoid Tumors

This type of cancer can develop in the child's soft tissues and other organs, such as the liver, lungs, and skin. It is also called malignant rhabdoid tumors (MRT) . "Malignant" means cancerous.

3. Atypical Teratoid Rhabdoid Tumors (ATRT)

This type of rhabdoid cancer develops in the child's central nervous system, the brain and spinal cord. In this type of cancer, the cancer cells first form in the child's brain or spinal cord. About 50% of these (ATRT) cancers develop in the cerebellum (which controls movement and balance) or the brainstem ( which controls things like breathing and heart rate).

Who is most likely to develop Rhabdoid Tumors?

It mainly affects infants and very young children , especially those between the ages of 11 and 18 months . Adults are much less likely to develop this disease.

This is so rare, some studies say it occurs in less than one in a million people.This cancer is said to occur in small amounts. That means it is a very rare condition.

Why do these rhabdoid tumors develop? What is the cause?

Most of the time, the main cause of rhabdoid cancer is a genetic mutation in the SMARCB1 gene . Imagine that there is a little guard in our body that controls the growth of cells. This SMARCB1 gene is a tumor suppressor gene . It produces a protein that controls cell growth. Like a traffic cop, it stops cells from dividing too quickly. So, if there is a defect, that is, a mutation, in this SMARCB1 gene, that control is lost and cells divide too quickly, leading to cancer . Rarely, these cancers can also develop due to a mutation in another tumor suppressor gene called SMARCA4. Although some children can inherit this mutated gene from their parents, most of the time these cancers occur due to a new genetic mutation that occurs without any family history . This means that even if no one in the family has had the mutation before, the child's cells may have this mutation by chance. When treating the child, a genetic evaluation may be done to look for this gene mutation.

What are the symptoms of Rhabdoid Tumor?

Symptoms can vary depending on the child's age and the location of the cancer . Symptoms usually begin in the area where the cancer is growing. These may include nerve damage, difficulty breathing, or a lump in the child's abdomen. Because this cancer spreads very quickly, symptoms can appear quickly. Other symptoms may include:

• Frequent fever.
• Headache .
• Blood in the urine ( Hematuria ) .
• Nausea and vomiting .
• Swollen lymph nodes.
• Constantly angry, restless (Irritability).
• Food is tasteless .
• Unusual drowsiness.
• In babies, the head size becomes larger.
• Loss of balance or difficulty walking.

If your child has one or more of these symptoms, it is very important to seek medical advice immediately.

How?How is this Rhabdoid Tumor diagnosed?

The doctor who examines your child will first do a physical exam and ask you about your child's symptoms. They will then order several tests to confirm the diagnosis. These may include:

• Ultrasound scan: This uses high-energy sound waves to create a picture (sonogram) of the baby's internal organs.
• CT scan: This uses X-rays and a computer to create a three-dimensional (3D) image of the child's soft tissues and bones.
• MRI scan: This uses a magnet, radio waves, and a computer to make detailed pictures of areas such as the child's brain and spinal cord.
• Neurological exam: This tests the child's brain, spinal cord, and nerve function.
• Lumbar puncture / Spinal tap: In this, a sample of cerebrospinal fluid (CSF) is taken from the child's spinal cord and examined under a microscope to see if there are any cancer cells.

However, these scans can show rhabdoid tumors that look like other types of cancer. Therefore, doctors often take tissue samples and order further tests. These tests include:

• Genetic testing: A sample of the child's blood or tissue is taken and tested for mutations in the genes `SMARCB1` and `SMARCA4`.
• Biopsy: In this procedure, the doctor uses a needle to take a small sample of the tumor. A pathologist then looks at it under a microscope to see if there are any cancer cells. If cancer cells are found, as much of the tumor as possible may be removed during the surgery or in a later surgery.
• Immunohistochemistry: This is a very specific test. It uses antibodies to look for certain markers/antigens in a sample of your child's tissue. These markers can help determine whether it is rhabdoid cancer or another type of cancer.

How is Rhabdoid Tumor treated?

A pediatric oncologist takes the lead in treating a child. He or she works with a team of other specialists to develop a treatment plan that best suits the child. Several different treatments may be used together to treat rhabdoid cancer. Because this cancer spreads so quickly, studies have shown that using multiple treatments together is more likely to kill cancer cells than using just one.

• Surgery: If the biopsy does not remove the cancer, the first step, if possible, is to perform surgery to remove as much of the cancer as possible, possibly all of it.
• Chemotherapy (Chemo): This involves giving drugs to stop the growth of cancer cells. These drugs kill some cancer cells and stop others from dividing. However, these drugs cannot tell the difference between good cells and cancer cells, so they can cause side effects.
• High-dose chemotherapy with stem cell transplant: High-dose chemotherapy can kill more cancer cells. However, it also kills healthy cells, such as blood-forming cells in the bone marrow. To address this, some of the child's bone marrow is taken and stored before high-dose chemotherapy is given. After treatment, the bone marrow is given back to the child to replace the destroyed cells.
• Radiation therapy: This uses high-energy X-rays to shrink or destroy cancer cells. However, because radiation therapy can affect a child's growth and brain development, the dose is adjusted according to the child's age and size.
• Clinical trials: The doctor may also suggest new clinical trials (new types of chemotherapy, targeted therapy, or immunotherapy drugs) to treat your child's cancer.

What are the possible side effects of the treatment?

Cancer treatments, especially chemotherapy, can cause side effects. This is because the drugs used to kill cancer cells also kill healthy cells. However, most side effects go away after treatment is stopped . You can work with your child's medical team to manage these side effects. Common side effects include:

• Hair loss `(Alopecia)` .
• The food is tasteless.
• Constipation.
• Diarrhea.
• Nausea and vomiting.
• Sores in the throat and mouth.
• Swelling `(Edema)` .
• Insomnia.
• Excessive fatigue .
• Memory problems.

Can Rhabdoid Tumors be prevented?

Unfortunately, these cancers are caused by genetic mutations, so there is no way to prevent them . However, if someone in your family has had rhabdoid cancer or other cancers, and you are expecting a child, it is a good idea to talk to your doctor about genetic counseling . Genetic counseling can help you understand your risk of having a genetic condition in your child.

Can Rhabdoid Tumor be cured? What will the child's future be like?

The doctor treating your child can give you the exact answer to this question. However,Unfortunately, most children with malignant rhabdoid tumor do not survive more than a few years. However, if the child is diagnosed after the age of 2 , the prognosis may be slightly better. The survival rate for this cancer depends on several factors:

• The child's age.
• Where the cancer is in the child's body.
• Whether the cancer has spread to other parts of the body.
• How much of the cancer was removed by surgery.
• How the child will respond to cancer treatments.

Because malignant rhabdoid tumors are so rare, life expectancy statistics are based on a very small number of patients. Therefore, a child's actual life expectancy can vary. Studies show that the five-year survival rate for rhabdoid tumor of the kidney (RTK) is between 20% and 25% . This means that between 20% and 25% of people diagnosed with RTK are still alive five years after diagnosis. The five-year survival rate for atypical teratoid rhabdoid tumors (ATRT) is between 32% and 50%.

It's normal to feel a great burden and sadness when you hear something like this. But remember, you are not alone.

What should I ask the doctor?

It's normal to have many questions when you find out your child has cancer. Here are some questions to remember to ask at that time:

• What kind of cancer does my child have?
• What is the stage of my child's cancer?
• What tests should my child have?
• What are the treatment options for my child's cancer?
• What are the chances of this treatment being successful?
• What clinical trials are available for my child to participate in?
• How do we know if the treatment is successful?

How is follow-up done during and after treatment?

Throughout your child's cancer treatment, some of the tests used to diagnose the disease will need to be repeated. These tests are used to measure how well the treatment is working. Based on the results of these tests, your medical team will work with you to decide what next steps you should take. This may include continuing, changing, or stopping treatment. After your child has finished cancer treatment, they will need to come back for periodic follow-up exams and tests . During these follow-ups, your doctor will check to see if your child's condition has improved or if the disease has returned. This follow-up may last for several years after treatment.

Finally, remember... (Take-Home Message)

We know it's heartbreaking to hear the news that your child has cancer. But it's important to remember that you are not alone.. The entire team that is treating your child, including doctors and nurses, is here to help you and your family during this difficult time. There are treatments for rhabdoid tumors, and there is hope for your child . Talk to your child's doctor about joining a cancer support group for parents or families. You can find great comfort and encouragement by sharing your story and hearing about other people's experiences.