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Neuroblastoma

What is neuroblastoma?

Highlights

  1. Neuroblastoma is a cancer that grows in the tissues that make up the sympathetic nervous system.
  2. It’s the most common type of cancer among infants. It’s usually diagnosed before children reach the age of 5.
  3. Neuroblastoma has four stages. Your child’s doctor will determine what treatments are needed based on the stage of your child’s cancer.

Your body’s nervous system is divided into the central nervous system, which includes your brain and spinal cord, and the peripheral nervous system. Your sympathetic nervous system is part of your peripheral nervous system. It helps carry messages from your brain to various parts of your body. It controls your:

  • fight-or-flight response
  • levels of certain hormones
  • digestion
  • heart rate
  • blood pressure

It also helps carry messages from your brain to various parts of your body.

Neuroblastoma is a cancer that develops in immature cells, or neurons, of the sympathetic nervous system. It develops as a solid tumor. It’s often found in the:

  • adrenal gland
  • pelvis
  • abdomen
  • neck
  • chest

If it progresses, it can spread to bones, lymph nodes, and skin.

While neuroblastoma is a rare cancer in general, it’s the most common type of cancer among infants. According to the Memorial Sloan Kettering Cancer Center, approximately 700 new cases of neuroblastoma are diagnosed each year in the United States. Most of them are diagnosed among young children. It’s usually diagnosed before they reach the age of 5.

Is neuroblastoma inherited?

Most cases of neuroblastoma aren’t inherited but are the result of a random gene mutation.  Approximately 1-2% of neuroblastomas are inherited in an autosomal dominant pattern. This means that you only need a gene from one parent to have the condition. Not all people who inherit such a gene develop neuroblastoma. This is known as “incomplete penetrance.” Experts believe that an additional mutation is required to develop neuroblastoma.

What are the symptoms of neuroblastoma?

Symptoms

Common  symptoms of neuroblastoma include:

  • a lump in the neck, chest, or abdomen
  • bulging eyes
  • dark circles under the eyes
  • abdominal swelling
  • bone pain
  • weakness in the upper or lower extremities
  • paralysis of, or an inability to move, the upper or lower extremities
  • painless, bluish swellings beneath the skin

Less common symptoms include:

  • fatigue
  • a fever
  • shortness of breath
  • a cough
  • high blood pressure
  • diarrhea
  • abnormal bleeding or bruising, including small, flat, red spots on the skin called petechiae
  • a rapid heart rate
  • excessive sweating
  • involuntary, uncontrolled movements of your eyes, feet, and legs

Many other conditions can cause these symptoms. They don’t necessarily point to a diagnosis of neuroblastoma.

How is neuroblastoma diagnosed?

Diagnosis

Due to the nonspecific nature of the symptoms of early neuroblastoma, the disease tends to have progressed to later stages before it’s diagnosed.

Your child’s doctor may diagnose neuroblastoma by using the following tests:

  • blood tests
  • urine tests
  • a bone marrow biopsy
  • a CT scan
  • an MRI scan
  • a positron emission tomography scan
  • a bone scan
  • an ultrasound

How is neuroblastoma staged?

 Type 1

After diagnosing neuroblastoma, your child’s doctor will stage their cancer. In other words, they’ll categorize the cancer based on its location and how far it has spread. The stage of your child’s cancer determines their course of treatment, which is why staging is crucial.

Neuroblastoma has four stages:

Stage 1

In stage 1, the tumor is in one area of your child’s body. It hasn’t spread yet, and your child’s doctor can remove it fairly easily.

Stage 2

In stage 2A, the tumor is in one area of your child’s body, but your child’s doctor can’t completely remove it during surgery. Cancer cells aren’t found in the local lymph nodes.

In stage 2B, the tumor is in one area of their body, and your child’s doctor can completely removed it during surgery. However, cancer cells are also found in the lymph nodes near your child’s tumor.

Stage 3

In stage 3, any of the following three situations may occur:

  • The tumor is still restricted to the area of your child’s body where it first developed. It’s only on one side of their body. However, cancer cells have been found in lymph nodes on the other side of their body.
  • The tumor is in the middle of your child’s body, and it’s spreading toward both sides of their body. This is due to growth of the tumor itself or the spread of cancer cells through your child’s lymph nodes.
  • The tumor can’t be entirely removed during surgery. It has also spread from one side of your child’s body to the other side. It may or may not have spread to nearby lymph nodes.

Stage 4

In stage 4, the tumor or cancer cells have spread to distant parts of your child’s body, such as the:

  • bone
  • liver
  • skin
  • distant lymph nodes
  • other organs

Stage 4S neuroblastoma behaves differently. It occurs when the following criteria are true:

  • Your child is younger than 1 year old.
  • The cancer is on one side of their body. It may have spread to lymph nodes on that side of their body but not the other side.
  • The tumor has spread to their liver, skin, or bone marrow.
  • Less than 10 percent of their bone marrow cells are cancerous.
  • The cancer hasn’t spread to their bones.

Risk level

Once your doctor has staged your child’s cancer, they’ll classify it as low, intermediate, or high risk. They’ll determine the risk level based on:

  • the stage of cancer
  • the tumor’s histology
  • the tumor’s biology
  • your child’s age

Low-risk and intermediate-risk neuroblastoma have a good chance of being fully cured. High-risk neuroblastoma is typically harder to cure.

How is neuroblastoma treated?

Treatment

The treatment for neuroblastoma depends on your child’s age and the stage of their cancer. It may include:

  • surgery
  • chemotherapy
  • radiation therapy
  • immunotherapy
  • a stem cell transplant

Many children with neuroblastoma will have more than one type of treatment. Treatment is typically done in stages and can last several years.

Chemotherapy

During chemotherapy, anticancer drugs are used to kill cancer cells. People usually receive these drugs intravenously, but your child can also get them orally, depending on the specific drug. Side effects can include:

  • hair loss
  • mouth sores
  • nausea
  • vomiting
  • a weakened immune system
  • fatigue

Side effects go away once your child finishes the treatment.

Radiation therapy

In radiation therapy, high-energy particles or rays, such as X-rays, are used to kill cancer cells. A machine typically aims the particles or rays at the affected area. This type of treatment can cause side effects, such as skin irritation, diarrhea, and fatigue.

Immunotherapy

Immunotherapy is also called biologic therapy. In this treatment, medications are used to stimulate your child’s immune system to fight disease.

Stem cell therapy

Stem cell therapy is also called a bone marrow transplant. After receiving high doses of chemotherapy or radiation therapy, replacement stem cells may be injected into your child’s bloodstream. Doctors typically reserve this treatment for high-risk children whose outlook with other treatment options is poor.

What is the outlook for children with neuroblastoma?

Outlook icon

Your child’s outlook will depend on their cancer’s stage and risk level. According to the American Cancer Society, children with low-risk neuroblastoma have a five-year survival rate that’s higher than 95 percent. Children with intermediate-risk neuroblastoma have a five-year survival rate of about 90 to 95 percent. Those in the high-risk group have a five-year survival rate of about 40 to 50 percent.

If their cancer treatment is successful, they’ll need to attend follow-up appointments to monitor for signs of relapse and potential side effects treatment. For low- and medium-risk cases, the risk of relapse is low. However, checkups are still important. Cancer treatment can cause long-term side effects. Not everyone will experience serious side effects, but they can occur. They can include:

  • developmental problems
  • learning difficulties
  • vision problems
  • seizures
  • muscle and bone issues
  • secondary cancers

Every child is different. Talk to your child’s doctor about the best treatment options for them. Ask them about the risks of specific treatment options, your child’s treatment schedule, and strategies to prevent, identify, and manage potential complications.

Many cancer centers and hospitals have support groups for children and families that are dealing with neuroblastoma or other cancers. They may provide helpful support and information.

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