Neuroblastoma is a type of cancer that usually affects children. It is very rare. Neuroblastoma begins in the early forms of the cells that make up the nerves. Sometimes, neuroblastoma grows slowly. In other cases, it can spread quickly. At times, it disappears on its own.

Neuroblastoma mostly affects babies or very young children. It usually does not affect children over the age of 10. Most cases involve children between 1 and 2 years old.

This cancer has usually begun to spread by the time doctors diagnose it. However, in younger children, the cancer cells can sometimes die by themselves. At times, the cells can also become benign, and therefore no longer harmful.

This article will explain what neuroblastoma is and describe its symptoms, stages, causes, diagnosis, and treatment.

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In children with neuroblastoma, immature nerve cells, called neuroblasts, become cancerous. This means they grow out of control and replicate.

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.

Most neuroblastomas start in the abdomen. Half begin in the adrenal gland. Most of the rest begin near the spine in the chest or neck, or in the pelvis.

Neuroblastoma is a very rare type of cancer, but it most commonly affects children. It makes up approximately 6% of all cancers in children.

According to the American Cancer Society, there are about 800 new cases of neuroblastoma each year in the United States.

Symptoms of neuroblastoma vary depending on where it is in the body.

General symptoms

Some of the more general symptoms may include:

Bone pain

Neuroblastoma may cause pain in the bones if it has spread there. This means a child might:

  • limp
  • not want to walk
  • not be able to walk

In the eyes

Caregivers may also notice that a child is experiencing symptoms relating to their eyes, including:

The actual cause of neuroblastoma is unknown.

According to St. Jude Children’s Research Hospital, neuroblastoma happens when nerve cells that are developing become cancerous. It can happen at the fetal stage or after birth.

A person can inherit neuroblastoma. This means biological parents with a personal or family history of the disease can pass it to their children.

According to the American Society of Clinical Oncology, a healthcare professional may use one of two staging systems:

The International Neuroblastoma Staging System Committee (INSS) system

  • Stage 1: Surgery can successfully remove the entire tumor. Local lymph nodes do not contain cancer, although ones attached to the tumor may.
  • Stage 2A: Surgery is unable to remove the entire tumor, but the tumor has not spread. Nearby lymph nodes do not contain cancer.
  • Stage 2B: Surgery may or may not be able to remove the entire tumor. The tumor has not spread, but nearby lymph nodes do contain cancer.
  • Stage 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.
  • Stage 4: The tumor has spread to lymph nodes in parts of the body away from its original site, or to the bones, bone marrow, liver, skin, and other organs.
  • Stage 4S: The physical tumor is only where it originally began, however, it has spread to the liver, skin, and/or bone marrow in children under 1. Usually, less than 10% of bone marrow cells show cancer.

The International Neuroblastoma Risk Group Staging System (INRGSS) system

Different countries use different staging systems, which means that it can be difficult to compare the results of international studies. Researchers developed this system to help with this issue.

The INRGSS uses image-defined risk factors (IDRFs), which are factors that could cause the tumor to be more difficult to remove. It involves four stages:

  • L1: The tumor has not spread into vital structures of the body, and is confined to one area, like the neck, chest, or stomach. There are no IDRFs.
  • L2: The tumor has not spread far from where it originated but does have at least one IDRF.
  • M: The tumor has spread to other parts of the body.
  • MS: The tumor has spread to only the skin, liver, and/or bone marrow in children younger than 18 months. Upon examination, less than 10% of marrow cells are cancerous.

The outlook for a child depends on the risk group that they are in. Doctors commonly use risk groups in the United States to categorize and predict how likely treatment is to 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 higher than 95%.
  • Intermediate-risk group: For children in the intermediate-risk group the 5-year survival rate is between 90% to 95%.
  • High-risk group: Children in the high-risk group have a 5-year survival rate of approximately 40% to 50%.

It is worth mentioning that in order to obtain these 5-year survival rates, researchers examine data from 5 years ago. This means that these rates may have improved, given the advances in medicine over that time period.

Neuroblastoma in adults is extremely rare. It affects 1 in 10 million adults per year.

Adults with neuroblastoma have a significantly worse outcome than children with neuroblastoma, with a 5-year survival rate average of 36.3%.

However, early diagnosis can help treatment be more effective.

One condition with similar signs and symptoms to neuroblastoma is nephroblastoma, also called Wilm’s tumor.

Wilm’s tumor is the most common renal cancer in children, as well as being the most common abdominal cancer in children. It usually affects children between 3 and 5 years old.

Wilm’s tumor may present with no symptoms. A mass will appear in an ultrasound scan.

There are approximately 650 new cases of Wilm’s tumor in the United States every year.

There are a few different treatment options for neuroblastoma, depending on the stage the cancer is at.

Treatment for neuroblastoma may include:

  • Surgery: This involves an operation to remove as much of the tumor as possible. If the tumor has not spread through the body, surgery could be the only treatment a child may need.
  • Chemotherapy: This involves using a mixture of drugs to kill the cancer cells. This is usually for children in the intermediate-risk or high-risk groups.
  • Radiation: This involves using high-energy rays such as X-rays to kill the cancer cells.

Imaging tests are important for diagnosing, staging, planning treatment, and following up with children with neuroblastoma. Types of imaging tests may include:

These scans can help doctors devise a treatment plan that best suits the individual child.

Sometimes, after treatment for neuroblastoma has ended, children may experience other health issues as a result.

They can include:

The risk of developing certain late effects varies depending on a variety of factors, including the specific drugs used during treatment, the dose of those drugs, and the age of the child during treatment.

A neuroblastoma diagnosis can be a very distressing experience for a child and caregiver. It is important to seek support, and there are many mental health resources available that can help.

Neuroblastoma is the most common cancer in infants. It shares a lot of symptoms with other conditions, but a person should take their child to the doctor if they notice that they are experiencing any possible symptoms, including bone pain or an inability to walk.

The outlook for a child with neuroblastoma depends on a number of different factors but can be higher than 95% in some cases.