Neuroblastoma in Children Homework Help
What is Neuroblastoma?
Neuroblastoma is a type of cancer of the nerve tissues and is uncommon. Neuroblastoma mostly affects children below 5 years. Common symptoms of this cancer include a lump in the belly, chest, or neck, decreased appetite, and fatigue.
Neuroblastoma is treated using surgery, radiation, and chemotherapy. The outlook depends on the age of the child, the stage of cancer, and the risk category. Neuroblastoma can be described as pediatric cancer that affects the nervous system and mostly grows in immature nerve tissues known as neuroblasts.
Neuroblastoma usually affects neuroblasts found in the adrenal glands found on top of the kidneys. Adrenal glands are responsible for producing hormones that are responsible for controlling automatic body functions like heart rate, digestion, breathing, and blood pressure.
In some cases, neuroblastoma can also develop in nerve tissue found in the abdomen, spinal cord, neck, or chest and may spread to other body parts. The outlook for children with this cancer depends on the stage of cancer, the location of the tumor, and the age of the child.
How common is Neuroblastoma?
Neuroblastoma is the most common cancer among children although it is very rare. In the United States, for instance, around 800 children are diagnosed with it every year. In most cases, neuroblastoma will occur in children below 5 years although it can also occur in unborn babies. This cancer is very rare in children who are 10 years or older.
What are the Stages of Neuroblastoma?
Neuroblastoma is classified based on how advanced the cancer is and how quickly it is growing and spreading. During classification, healthcare providers also consider if cancer has metastasized or spread to other parts of the body.
Healthcare providers will use such information to determine the risk level of cancer. Thereafter, they can be able to choose the appropriate treatment. In the past, healthcare providers determined the stage of cancer by finding out how much of it was found in the body after surgery.
Currently, the stage of neuroblastoma is determined using the International Neuroblastoma Risk Group Staging System. This method determines the stage of the cancer by focusing on how much it has spread in the initial imaging studies known as image-defined risk factors. These images can be either on MRI or Ct scan.
The International Neuroblastoma Risk Group Staging System stages of neuroblastoma include:
- Stage L1 – this stage has the lowest risk. Tumors in this stage have not spread and are confined to one compartment of the body. Tumors in this stage do not involve vita structures of the body. There are no image-defined risk factors present at this stage.
- Stage L2 – in this stage, the cancer is only on one body compartment although its cells can spread to nearing lymph nodes. This stage of neuroblastoma involves vital structures of the body. Tumors will usually be wrapped around large blood vessels. At this stage, there is one image-defined risk factor.
- Stage M – at this stage, the cancer cells have spread to more than one compartment of the body also known as distance metastatic disease. This stage of neuroblastoma is the stage with the highest risk.
- Stage MS – this category of neuroblastoma affects children who are younger than 18 months. At this stage, cancer cells have metastasized or spread to either the liver, bone marrow, or skin only. The prognosis for children with this stage of Neuroblastoma is excellent. This stage is considered a low-risk disease.
What Causes Neuroblastoma in Children?
Neuroblastoma occurs when neuroblasts which are immature nerve tissues grow out of control. Cells of these tissues become abnormal and continue to grow and divide to form a tumor.
These cells grow and divide uncontrollably due to a genetic mutation or a change in the genes of the neuroblast. It is unclear what causes this genetic mutation. However, children with a family history of this cancer are more likely to develop this cancer compared to those with no history of it.
However, it is important to note that almost 99% of the time, cases of neuroblastoma are not inherited. Children who are born with congenital anomalies also known as birth defects are more likely to develop neuroblastoma.
What are the Symptoms of Neuroblastoma?
Symptoms of neuroblastoma range from mild to severe. Symptoms of neuroblastoma vary depending on the stage of the disease and the location of the tumor. By the time the symptoms appear, cancer will have spread to other parts of the body.
Symptoms of neuroblastoma include:
- a lump or bump in the chest, pelvis, neck, abdomen, or several lumps under the skin that are blue or purple in infants.
- painful bloated tummy
- trouble breathing especially in young babies
- bulging eyes or dark circles under the eyes. This may look like the child has a black eye.
- weakness, paralysis in the feet and legs, and movement problems.
- fatigue, cough, and fever
- Pale skin which is a sign of anemia
- diarrhea, constipation, loss of appetite, or upset stomach
Symptoms of neuroblastoma that are likely to appear as the disease progresses include:
- shortness of breath
- uncontrollable eye movements or eyes that dart around quickly
- pain in the bones, legs, or back
- Horner’s syndrome which causes small pupils, droopy eyelid, and sweating on one side of the face.
- problems with balance, coordination, and movement
- high blood pressure
How is Neuroblastoma Diagnosed?
Most children with neuroblastoma will receive a diagnosis before they celebrate their 5th birthday. Neuroblastoma is the most common cancer in infants. In some cases, neuroblastoma can be diagnosed during a prenatal ultrasound.
During diagnosis, the healthcare provider will conduct a neurological and physical examination. A neurological exam will help check the child’s reflexes, coordination, and nerve function.
Additionally, the doctor may order additional tests to confirm the diagnosis and to determine if cancer has metastasized. Additional tests done include;
- blood and urine tests – a complete blood count will be ordered to check for abnormalities in the blood and anemia. Blood chemistry tests are also done to detect substances in the blood that may indicate the presence of cancer or to measure hormone levels. A urine test is done to measure the number of chemicals in the body of the child.
- bone marrow biopsy – a bone marrow biopsy will the bone marrow, blood, and bones for any signs of cancer. Bone marrow is the sponge-like tissue found at the center of the large bones. This is where blood cells form.
- biopsy – a biopsy will involve removing a tissue sample from the child and sending it to the laboratory. In the laboratory, the tissue from the tumor will be examined under a microscope. Several tests will also be done on the sample as a way of confirming the diagnosis of neuroblastoma. Specialized biopsy tests will look for specific changes or alterations of the tumor which can help determine the risk category of the child and their treatment plan.
- MRI scan – a magnet and radio waves are used to produce images of soft tissues in an MRI scan.
- CT scan – during a CT scan, the doctor will inject a special dye into the child’s vein. They will then perform several X-rays. The dye is used to make it easier to see tumors and tissues.
- Ultrasound – during an ultrasound, high-frequency sound waves are used to create images of soft tissues.
- methyliodobenzylguanine scan – this is an imaging test that uses a safe radioactive chemical known as 123-iodinated MIBG radiotracer. The radiotracer is inserted in the child’s vein and the next day a special type of scanner is used to take pictures of the child’s organs.
This compound is very specific for neuroblastoma cells. The radiotracer goes to any area where neuroblastoma is. This nuclear medicine helps the doctor determine if the neuroblastoma has spread or not.
However, neuroblastoma tumors in some patients do not take up this compound. In such cases, a PET scan is done to locate all areas of active neuroblastoma cells.
- X-ray – an abdominal or chest x-ray can help locate the tumor and show how the tumor is affecting other tissues in the body. An x-ray gives a less detailed image of how the cancer is affecting the body.
How is Neuroblastoma in Children Treated?
Treatment for neuroblastoma will depend on the age of the child, the location of the tumor and the stage of the disease, and its category. Parents and doctors work together to determine the most appropriate treatment plan for the child based on the risk category.
Risk categories for neuroblastoma include:
- Low-risk neuroblastoma – children in this category do not need immediate treatment. For children below 6 months, some tumors go away on their own without any treatment.
other children in this category may need chemotherapy or surgery to remove the tumor or both. The health of the child will be monitored closely.
- Intermediate risk neuroblastoma – children in this category will need surgery to remove the tumor. Cancer cells that may have spread to nearing lymph nodes are also removed surgically. Once the surgery is done, chemotherapy treatments are done.
In some cases, however, chemotherapy is done before surgery to shrink the tumor to improve the chances of the surgery being successful. The number of chemotherapy sessions done depends on the tumor’s response to treatment and certain risk factors.
- High-risk neuroblastoma – high-risk neuroblastoma is often treated with a combination of chemotherapy, high dose chemotherapy with stem cell rescue, surgery, immunotherapy, and radiation. After treatment, children in this category will need to take medications for several months.
Those with extra copies of the MYCN gene will need high-risk neuroblastoma treatment no matter the stage they are in. Children who have more of this gene will be treated using the most aggressive treatment because the MYCN gene promotes the abnormal growth of cells. This means that it can cause tumors to grow and spread throughout the body very fast.
Treatments for Neuroblastoma
The treatment option recommended by the doctor will depend on several factors. Doctors may recommend radiation, chemotherapy, surgery and other treatments to treat neuroblastoma.
Treatments for neuroblastoma include:
- Chemotherapy – chemotherapy is done to stop the multiplication of the cancer cells. Chemotherapy drugs are injected into the vein of the child. Chemotherapy will require treatments for several weeks or months. The length of the treatment, as well as the type of chemotherapy regimen used, depends on the risk category of the child.
- Radiation – radiation kills cancer cells and stops their multiplication. High levels of radiation are used to target cancer cells in radiation therapy. Radiation is often used by radiation oncologists to prevent the cancer from coming back after treatment. Radiation is mostly used in patients with high-risk neuroblastoma. Radiation is not needed for intermediate-risk and low-risk neuroblastoma patients.
- Surgery – an incision is done to remove the tumor. However, it may be impossible to remove an entire tumor. It is for this reason that chemotherapy is done before or after surgery in some children to shrink or destroy remaining cancer cells respectively.
- Stem cell transplant – in stem cell transplant, a stem cell is removed from the child’s bloodstream and is stored in the freezer during the first rounds of chemotherapy. After high-dose chemotherapy, the doctor will inject back the stem cell into the child’s bloodstream. The injected stem cell will travel to the bone marrow to destroy the stem cells that were destroyed during treatment. The new cells are then used to rebuild the immune system.
- Immunotherapy – cancer cells can remain even after radiation and chemotherapy. Immunotherapy is used in such cases to train the body to attack abnormal cells. Antibodies are injected through the vein. Currently, immunotherapy medications are used to target a protein called GD2 that is found on the outside of the neuroblastoma cell. Antibodies locate these GD2 proteins and attach to them. The immune system is signaled to destroy them.
- Iodine 131-MIBG therapy – this treatment involves using radiation to target tumor cells. Radioactive iodine is injected into the child’s bloodstream through a vein. The radioactive iodine then uses radiation to kill tumor cells. This treatment option is mostly used in children whose neuroblastoma has refused to go away or has come back. This treatment method is being studied because it is being considered to be the frontline therapy for high-risk neuroblastoma cases.
- Medications – in some cases, drugs may be recommended to stop the multiplication of cancer cells, destroy them and reduce blood flow to the tumor. Some of these medications are given after treatment to prevent cancer from coming back. Other drugs that are prescribed are used to work on tumors that do not go away even after standard treatment.
Prevention of Neuroblastoma in Children
Is it possible to prevent neuroblastoma?
It is not possible to prevent neuroblastoma. Parents who have a family history of neuroblastoma or have had neuroblastoma as children should talk to the doctor. It is recommended that genetic counseling is done for all children who have had neuroblastoma to determine if genetic testing is needed.
Genetic testing helps tell if the child has gene markers for inherited neuroblastoma. Inherited neuroblastoma is very rare. Some gene mutations that are passed down through families are likely to increase the risk of neuroblastoma.
For instance, Li Fraumeni syndrome increases the risk for neuroblastoma and other conditions.
What is the Outlook/Prognosis for Children with Neuroblastoma?
The outlook for children with neuroblastoma is different. Cancer outlook is measured by the 5-year survival rate. Children who are younger and with a low to intermediate-risk neuroblastoma have a good prognosis with a 90-95% rate of survival.
The survival rate and prognosis for older children with high-risk neuroblastoma is around 60%. Researchers and healthcare providers are researching to come up with better treatments for this category of patients and the studies look promising.
The prognosis for neuroblastoma depends on the following factors:
- the biological makeup of the tumor
- the age of the child at the time of the diagnosis
- if the tumor has spread to the lymph nodes or other parts of the body
Living with neuroblastoma
Parents whose children show any signs and symptoms of neuroblastoma should take their children to the doctor immediately. The prognosis of the child depends on how early the diagnosis is made and how early the treatment begins.
A cancer diagnosis can change the life of the entire family. However, it is not the end. There is hope. Today, researchers and healthcare providers are working to come up with treatments that are likely to greatly improve the prognosis for kids with neuroblastoma.
Researchers have performed several clinical trials with very promising outcomes. More research is still going on. Parents whose children are diagnosed with neuroblastoma should consider joining a cancer support group for emotional support. Sharing experiences and stories of hope can help them feel supported and encouraged.
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