Neuroblastoma

1 What is Neuroblastoma?

Neuroblastoma is a cancer of primitive nerve cells (neuroblasts) commonly originating in the adrenal glands, situated above the kidneys, and less commonly in other abdominal areas, chest, neck and spinal areas.

This rare cancer usually affects children aged 5 or younger.

Treatment is required when neuroblastoma does not resolve on its own. The choice of treatment approach is determined by a variety of factors.

2 Symptoms

Depending upon the body part affected by neuroblastoma, you may experience different signs and symptoms that include: 

Neuroblastoma in the abdomen

  • Abdominal pain
  • A lump present below the skin that isn't painful when touched
  • Altered bowel habits, such as diarrhea or constipation

Neuroblastoma in the chest 

  • Whistling sound during exhalation (Wheezing)
  • Pain in chest
  • Eye changes such as sagging eyelids (drooping eyelids) and unequal pupil size

Neuroblastoma can also cause following signs and symptoms: 

  • Mass of tissue beneath the skin
  • Bulging of eyeballs (proptosis)
  • Undereye dark circles that appear like bruises
  • Back pain
  • Fever
  • Unintended weight loss
  • Bone pain

When to see a doctor?

Make an appointment with your child's doctor if your child has any signs or symptoms of neuroblastoma. 

3 Causes

It is unclear what the exact cause of Neuroblastoma is.

DNA mutations can cause “out-of-control” growth and multiplication of cells. Unlike normal cells, these abnormal cells do not die and accumulate to form tumors.

As the name suggests, neuroblastoma originates in the early nerve cells called neuroblasts, in the unborn baby. Normally, these neuroblasts mature into nerve cells and fibers, and cells of adrenal glands.

While most neuroblasts mature by the time of birth, some may still persist leading to neuroblastoma.

The cause of DNA mutation in the neuroblasts is still a mystery.

4 Making a Diagnosis

Diagnosis of neuroblastoma include the following tests and procedures: 

  • Physical exam: It involves simple tests to detect signs and symptoms of the disease. In some cases, you may have to explain habits and behaviors.
  • Urine and blood tests: These tests can also help to determine the cause of your child's signs and symptoms. Higher levels of catecholamines can be detected by urinalysis. 
  • Imaging tests: Imaging tests, such as X-ray, ultrasound, computerized tomography (CT) scan, metaiodobenzylguanidine (MIBG) scan and magnetic resonance imaging (MRI) help reveal tumor, if any. 
  • Biopsy: A sample of tissue from tumor may be obtained for lab analysis. The lab analysis can detect the types of cell present in the tumor the genetic abnormalities of the cancer cells. Both of these information are useful in developing appropriate and individualized treatment plan.
  • Bone marrow biopsy: Bone marrow testing and bone marrow aspiration may be performed to check whether the neuroblastoma has metastasized to bone marrow. In this procedure, a fine needle is inserted into your child's hipbone or lower back to aspirate (draw out) the bone marrow.

Staging

Staging your child’s cancer helps to select appropriate treatment option. Imaging tests, such as X-rays, bone scans, and CT, MRI and MIBG scans, are opted for determining the stage of cancer. 

Stages of neuroblastoma are:

  • Stage I: Neuroblastoma is limited to certain area and entire tumor can be surgically removed. Cancer cells may be present in lymph nodes connected to the tumor but not in other lymph nodes.
  • Stage IIA: Neuroblastoma is confined to one area but entire tumor cannot be removed surgically.
  • Stage IIB: Neuroblastoma is localized and complete surgical removal of tumor may or may not be possible. The cancer has spread to neighboring lymph node. 
  • Stage III: Complete removal of tumor by surgery may be impossible. The tumor may or may not have spread to nearby lymph nodes and is larger in size. 
  • Stage IV: Neuroblastoma has metastasized (spread) to distant lymph nodes or different parts of the body.
  • Stage IVS: This stage is diagnosed in children younger than 1 year. In this stage, the cancer has metastasized to other parts of the body mostly to skin, liver or bone marrow. The babies are likely to recover from this condition. The cancer, sometimes, may resolve on its own without any treatment. 

5 Treatment

The treatment plan for neuroblastoma is based on the risk category.

  • Surgery: Surgical removal of tumor may be the only treatment opted for children with low risk neuroblastoma. Location and size of tumor decide whether the tumor can be completely removed. Removal of tumors attached to vital organs, such as lungs or spinal cord, may cause complications. Doctors may try to remove tumor as much as possible in children with intermediate and high risk neuroblastoma. Surgery may be followed by other treatments, such as chemotherapy and radiation, to destroy remaining cancer cells.
  • Chemotherapy: Chemicals used in chemotherapy kill cancer cells. Chemotherapy destroys rapidly dividing cells. Children may be given combination of chemotherapy agents prior to surgery to increase the chance of complete removal of cancer cells. Chemotherapy in high doses is given to children with high-risk neuroblastoma to kill the cancer cells. Chemotherapy may be given prior to surgery and bone marrow stem cell transplant.  
  • Radiation therapy: Radiation therapy utilizes high-energy radiation, such as X-rays, to kill cancer cells. Radiation therapy for children with low-risk or intermediate-risk neuroblastoma may be opted if surgery and chemotherapy fail to treat the condition. It may be given to children who have high-risk neuroblastoma after chemotherapy and surgery, to prevent the recurrence of cancer. The side effects depend on the area where radiation is aimed and the dose of radiation administered.
  • Stem cell transplant: This option is chosen for children with high-risk neuroblastoma (autologous stem cell transplant). In this procedure, stems cells from your child's blood is collected and stored. Afterwards, high doses of chemotherapy are given to your child to kill remaining cancer cell. The stored stem cells are then re-injected into your child's body so that they can form healthy blood cells. 
  • Immunotherapy: Immunotherapy uses drugs that stimulate your body's immune system against cancer cells. Immunotherapy may be used in treatment of children who suffer from high-risk neuroblastoma.
  • Newer treatments: The newer form of radiation therapy uses radioactive form of the chemical metaiodobenzylguanidine (MIBG). MIBG when injected are taken by neuroblastoma cells where they release radiation to kill cancer cells. It is used to treat high risk neuroblastoma. MIBG can be used in combination with chemotherapy or stem cell transplant. Your child will be hospitalized for few days after receiving MIBG. 

Your child's age, the stage of cancer, types of cells involved in cancer and presence of any genetic abnormalities are used to classify the cancer as low risk, intermediate risk or high risk. 

6 Lifestyle And Coping

Knowing that your child has neuroblastoma can be devastating. Following tips might be useful to cope with the anxiety and stress:

  • Expand and update your knowledge on your child's condition. Discuss with your child's health care team about his/her condition. Make sure you ask questions to your child's doctor if you have any queries. 
  • Stay close to your friends and family. 
  • Join support groups in your community or online, if any. 
  • Discover your own ways to comfort your child during hospital visits and treatment. Your child's health care team might suggest you ways to comfort your child.
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