Neuroblastoma

Neuroblastoma almost always develops in infants and young children. It rarely occurs in children over 10 years of age.

Neuroblastoma begins in neuroblasts, which are immature cells in the sympathetic nervous system. The sympathetic nervous system is responsible for actions such as blushing, increasing heart rate or your pupils’ dilating. These are known as involuntary actions.

This solid tumour can occur anywhere in the body, but it usually starts in the abdomen, often in one of the two adrenal glands, which are just above the kidneys. Other common sites are the chest, neck or pelvis.

About this Cancer

  • Neuroblastoma statistics are often included with statistics on endocrine cancers, and may not be an exact reflection of neuroblastoma incidence and mortality rate. 
  • Between 2012 and 2016, 160 children in Canada were diagnosed with endocrine system cancers.
  • Between 2013 and 2017, 57 children in Canada died from endocrine system cancers. 
  • For more information, check out Neuroblastoma statistics | Canadian Cancer Society
  • Neuroblastoma almost always develops in infants and young children. It rarely occurs in children over 10 years of age.
  • Neuroblastoma begins in neuroblasts, which are immature cells in the sympathetic nervous system. The sympathetic nervous system is responsible for actions such as blushing, increasing heart rate or your pupils’ dilating. These are known as involuntary actions.
  • This solid tumour can occur anywhere in the body, but it usually starts in the abdomen, often in one of the two adrenal glands, which are just above the kidneys. Other common sites are the chest, neck or pelvis.
  • A mass in the abdomen
  • A sensation of abdominal fullness or bloating
  • Bulging of the eyes
  • Discoloration/darkening under the eyes
  • Pain in the bones
  • Reduced levels of red blood cells, white blood cells, and platelets
  • Skin nodules or lesions, occasionally with a bluish hue
  • Loose stools or diarrhea
  • Elevated blood pressure 
  • Fever
  • Anemia
  • Impaired sensation or mobility in certain body areas
  • A drooping eyelid accompanied by a small pupil

Note that neuroblastoma may initially be asymptomatic. Symptoms typically arise as the tumor grows, varying by its location. Other health conditions may cause the same symptoms as neuroblastoma. 

For more information, please consult: Symptoms of neuroblastoma | Canadian Cancer Society

  • Family history: Around 1% to 2% of children with neuroblastoma have a family history of the disease. The risk is greatest for siblings or identical twins of affected children. In most cases with a family history, genetic mutations in the anaplastic lymphoma kinase (ALK) gene are present. 
  • Genetic conditions: Children that have the following genetic conditions are at increased risk for developing neuroblastoma:
    • Hirshsprung’s disease: a disorder where certain nerves are absent in a section of the intestines, causing the large intestine to malfunction and potentially become obstructed.
    • Congenital central hypoventilation syndrome (CCHS): This is a rare condition that impairs breathing, causing shallow breaths, particularly during sleep.
    • Neurofibromatosis type 1: This is a condition that disrupts nerve cell growth and development, leading to tumors in the nerves and skin. Children with this condition face an increased risk of cancers such as neuroblastoma. 
    • Beckwith-Wiedemann Syndrome: This condition alters the growth of various body parts. Affected children are born significantly larger than average and experience rapid growth and weight gain during childhood. They also have an increased risk of certain cancers, including neuroblastoma.
    • Li-Fraumeni syndrome: This condition is associated with an increased risk of developing various types of cancer, including neuroblastoma. 
    • Costello syndrome: This syndrome impacts multiple areas of the body. It affects development, causes intellectual disability, distinct facial features, extra skin folds, and flexible joints. It also increases the risk of cancers, including neuroblastoma.

For more information: Risk factors for neuroblastoma | Canadian Cancer Society

  • Health history and physical exam
  • Urine test
  • Blood chemistry tests: Tests that measure specific chemicals in the blood. These tests are used to show how well specific organs are functioning, and they can identify abnormalities.
  • Complete blood count: A complete blood count is used to measure the number and quality of white blood cells, red blood cells, and platelets. It can be used to check for anemia, and the clotting ability of blood. 
  • Ultrasound: An ultrasound uses high frequency sound waves to create images of the body, helping to detect abdominal tumors or the cause of swelling.
  • MRI: An MRI uses strong magnetic forces to produce cross sectional iamges that are transformed into 3D pictures, and it can be used to locate tumors and determine their size.
  • CT scan: A CT scan produces cross-sectional images of the body and can be used to determine the size of the tumor and if it is close to/touching vital organs. 
  • MIBG scan: An MIBG scan is a nuclear imaging test where metaiodobenzylguanidine (MBG), containing radioactive material, is injected and absorbed by neuroendocrine cells. It helps identify tumors, assess disease spread, and monitor treatment response.
  • PET scan: A PET scan uses radioactive materials such as radiopharmaceuticals to observe changes in the metabolic activity of body tissues. It is used to look for neuroblastoma that does not respond to MIBG. 
  • Bone scan
  • X-ray: An x-ray uses a minimal dose of radiation to produce images of the body, and for neuroblastoma, it can be used to look for a mass in the chest or abdomen. 
  • Biopsy: A biopsy involves the removal of tissues or cells from the body for further assessment and to determine if cancer cells are present.
  • Molecular genetic methods: Molecular genetic tests assess for abnormalities in the chromosome, for example, duplications, translocations, mutations and deletions. For neuroblastoma, they are helpful for determining health outcomes and the treatment needed.
  • Watchful waiting can be offered to patients with neuroblastoma. This option allows healthcare workers to observe the cancer closely and check to see if it is growing before offering alternative treatments.
  • Low-risk and intermediate risk neuroblastoma can be treated with surgery, chemotherapy and radiation therapy
  • High risk neuroblastoma can be treated with chemotherapy, surgery, stem cell transplant, radiation, retinoids, and immunotherapy 
  • 4S neuroblastoma: There is no standard treatment for this stage of neuroblastoma. In Most children may not need to have any treatment
  • Treatment for recurrent neuroblastoma is based on the risk group that is given to the cancer during the initial diagnosis

For more information, please consult Treatments for neuroblastoma | Canadian Cancer Society

Resources

Neuroblastoma Cancer Groups

  • Neuroblastoma Canada: Neuroblastoma Canada is a national community – based organization dedicated to unite every Canadian Neuroblastoma family through open discussion, online resources, and supportive activities.
  • The James Fund for Neuroblastoma Research: The James Fund for Neuroblastoma Research is a not-for-profit which raises funds for neuroblastoma research, awareness about the disease, and provides support to neuroblastoma families. Their mission is “inspiring change by sharing the unfolding story of our search to find a cure for neuroblastoma by raising funds while supporting families suffering the same pain.”