Neuroblastoma is a cancer that usually affects children. It’s very rare. Neuroblastoma starts in the early forms of the cells that make up the nerves. Sometimes neuroblastoma grows slowly. In other cases, it can spread quickly. Sometimes it goes away on its own.
Neuroblastoma mainly affects babies or very young children. It does not usually occur in children over the age of 10. In most cases, children are between 1 and 2 years old.
This cancer usually starts to spread by the time the doctors make the diagnosis. However, in younger children, the cancer cells can sometimes die on their own. Sometimes the cells can also become benign and therefore no longer be harmful.
This article explains what neuroblastoma is and describes its symptoms, stages, causes, diagnosis and treatment.
In children with neuroblastoma, immature nerve cells called neuroblasts become cancerous. This means that they get out of control and multiply.
Neuroblastoma can affect any part of the sympathetic nervous system. The sympathetic nervous system is part of the autonomic nervous system, which the body uses to regulate many of its most crucial functions.
Read more about the autonomic and sympathetic nervous system here.
Most neuroblastomas start in the abdomen. Half starts in the adrenal gland. Most of the rest starts at the spine in the chest or neck, or in the pelvis.
Neuroblastoma is a very rare cancer, but it usually affects children. It makes up about 6% of all cancers in children.
According to the American Cancer Society, there are about 800 new cases of neuroblastoma in the United States every year.
Symptoms of neuroblastoma vary depending on where it is located in the body.
Some of the more common symptoms can include:
Neuroblastoma can cause bone pain if it has spread there. This means that a child:
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In the eyes
Health care providers may also notice that a child is experiencing symptoms related to their eyes, including:
According to the American Society of Clinical Oncology, a health care provider can use one of two staging systems:
The International Neuroblastoma Staging System Committee (INSS) system
Phase 1: Surgery can successfully remove the entire tumor. Local lymph nodes do not contain cancer, although those attached to the tumor can.Step 2A: Surgery cannot remove the entire tumor, but the tumor has not spread. Nearby lymph nodes do not contain cancer.Step 2B: Surgery may or may not be able to remove the entire tumor. The tumor has not spread, but nearby lymph nodes contain cancer. Step 3: Surgery cannot remove the tumor, and cancer has spread to nearby lymph nodes or other areas near the tumor. Cancer has not spread to other parts of the body.Phase 4: The tumor has spread to lymph nodes in parts of the body, away from its original location, or to the bones, bone marrow, liver, skin, and other organs.Phase 4S: The physical tumor is only where it originally started, but it has spread to the liver, skin, and / or bone marrow in children under 1 year of age. Usually less than 10% of the bone marrow cells show cancer.
The International Neuroblastoma Risk Group Staging System (INRGSS) system
Different countries use different staging systems, which means it can be difficult to compare the results of international studies. Researchers have developed this system to help with this problem.
The INRGSS uses image-defined risk factors (IDRFs), which are factors that can make the tumor more difficult to remove. It includes four stages:
L1: The tumor has not spread to vital structures of the body and is limited to one area, such as the neck, chest, or stomach. There are no IDRFs.L2: The tumor has spread not far from its origin, but it does have at least one IDRF.M: The tumor has spread to other parts of the body.MRS: The tumor has spread to the skin, liver, and / or bone marrow only in children less than 18 months of age. In research less than 10% of the marrow cells are cancerous.
Read more about cancer staging here.
The outlook for a child depends on the risk group they are in. In the United States, doctors often use risk groups to categorize and predict the likelihood that treatment will cure a child.
Doctors base risk groups on a number of different factors, including the stage of the neuroblastoma and the age of the child at the time of diagnosis. A person can talk to a doctor to discuss individual risk groups.
Low risk group: In the low-risk group, children have a 5-year survival rate that is greater than 95%.Intermediate risk group: For children in the middle risk group, the survival rate after 5 years is between 90% and 95%.High risk group: Children in the high-risk group have a 5-year survival rate of approximately 40% to 50%.
It is worth noting that researchers are examining data from 5 years ago to obtain these 5-year survival rates. This means that these rates may have improved, given the advancements in medicine over that period.
Neuroblastoma in adults is extremely rare. It affects 1 in 10 million adults each year.
Adults with neuroblastoma have a significantly worse outcome than children with neuroblastoma, with a mean survival rate of 36.3% of 5 years.
However, early diagnosis can make treatment more effective.
A condition with similar signs and symptoms to neuroblastoma is nephroblastoma, also called Wilms tumor.
Wilm’s tumor is the most common childhood kidney cancer, but also the most common childhood abdominal cancer. It usually affects children between the ages of 3 and 5.
Wilm’s tumor can present without symptoms. A mass will show up on an ultrasound.
There are about 650 new cases of Wilms tumor in the United States every year.
Read more about Wilm’s tumor here.
There are a few different treatment options for neuroblastoma, depending on what stage the cancer is in.
Treatment for neuroblastoma can include:
Surgery: It is an operation to remove as much of the tumor as possible. If the tumor has not spread throughout the body, surgery may be the only treatment a child needs.Chemotherapy: This involves using a mixture of drugs to kill the cancer cells. This is usually for children in the intermediate or high risk groups.Radiation: This includes using high energy rays such as X-rays to kill the cancer cells.
Imaging tests are important for diagnosing, staging, planning treatment and monitoring children with neuroblastoma. Types of imaging tests can include:
These scans can help doctors design a treatment plan that is best for the individual child.
Sometimes, after treatment for neuroblastoma ends, children can experience other health problems as a result.
They can be:
The risk of developing certain late effects varies depending on a number of factors, including the specific medications used during treatment, the dose of those medications, and the age of the child during treatment.
A diagnosis of neuroblastoma can be a very distressing experience for a child and caregiver. Seeking support is important, and there are many mental health resources that can help.
Learn more about mental health resources for children here.
Neuroblastoma is the most common cancer in infants. It shares many symptoms with other conditions, but a person should take their child to the doctor if they find themselves experiencing possible symptoms, including bone pain or inability to walk.
The outlook for a child with neuroblastoma depends on a number of different factors, but can exceed 95% in some cases.