Overview:

Neuroblastoma is a rare cancer that originates in the nervous system.1 It starts in early unmature nerve cells that are most often found in the embryo or foetus2 and is the most common extracranial solid tumour diagnosed in children under 15 years of age, comprising around 7% of all childhood cancers3.

It affects approximately 1,600 babies and young children every year across the EU4 and US5 (800 in each region respectively).

Symptoms: 

Early symptoms can be vague and hard to spot, and so may be mistaken for more common childhood conditions.6 Symptoms vary depending on where the tumour is in the body and if it has spread.7 65% of neuroblastomas develop in the abdomen, for example the adrenal gland and kidney.8 A common symptom is a lump in the abdomen, that can cause swelling, pain, constipation or diarrhoea. Other symptoms include8:

  • high blood pressure
  • breathlessness and difficulty swallowing

If the lump is located in the chest may cause signs and symptoms such as:

  • wheezing
  • chest pain

In some cases, neuroblastoma can affect the spinal cord.8 This can cause:

  • numbress
  • weakness
  • loss of movement in the lower part of the body

In some cases, neuroblastoma can affect the spinal cord.8 This can cause:

  • lump of tissue under the skin
  • eyeballs that seem to protrude from the sockets (proptosis)
  • dark circles, similar to bruises, around the eyes
  • back pain
  • fever
  • unexplained weight loss
  • bone pain

Diagnosis

Most neuroblastomas are diagnosed in babies or children under five years old.9 Researchers do not yet know the cause of neuroblastoma. Neuroblastomas are usually picked up when a child is brought to their doctor because of the symptoms they are experiencing. 1,10

In 50% of patients, the cancer has already spread at diagnosis.11 Delays in diagnosis are often due to the complex nature of the cancer, for example the wide variety of symptoms.12

Around 50% of patients are diagnosed with high-risk neuroblastoma and this has the worst prognosis.8 Normally, high-risk neuroblastoma means the cancer has spread. Patients will need an intensive treatment approach, often using a combination of different therapies.13

 

References:
1 Pastor ER, Mousa SA. 2019. Current management of neuroblastoma and future direction. Critical Reviews in Oncology/Hematology. 138:38-43.
2 American Cancer Society. What is neuroblastoma? Available at: https://www.cancer.org/cancer/neuroblastoma/about/what-is-neuroblastoma.html 
3 Nadja C. Colon et al. Neuroblastoma. 2011; 58(1): 297–311. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3668791/
4 Gatta G et al. European Journal of Cancer. 2012; 48, 1425-1433. Note: 1.8 cases of neuroblastoma per million were estimated every year in EU 27. With the current population estimated at 448 million this would equate to 806.4 patients 
5 American Cancer Society. Key Statistics About Neuroblastoma. Available at: https://www.cancer.org/cancer/neuroblastoma/about/key-statistics.html [November 2020]
6 NHS. Neuroblastoma. Available at: https://www.nhs.uk/conditions/neuroblastoma/ Last accessed November 2021
7 Swift, CC et al. Updates in Diagnosis, Management, and Treatment of Neuroblastoma. RadioGraphics, 2018. Volume 38, Issue 2, Pages 566 -580
8 Medscape. Neuroblastoma: Practice Essentials, Background, History of the Procedure. Available at: https://emedicine.medscape.com/article/439263-overview#a10 
9 Shohet J and Foster J. 2017. Neuroblastoma. BMJ. 357:j1863 doi:10.1136/bmj.j1863.
10 American Cancer Society. Tests for neuroblastoma. Available at: https://www.cancer.org/cancer/neuroblastoma/detection-diagnosis-staging/how-diagnosed.html 
11 Ward, E et al. 2014. Childhood and adolescent cancer statistics. CA: A Cancer Journal for Clinicians. 64(2): 83–103.
12 Tolbert, V.P. and Matthay, K.K. 2018. Neuroblastoma: clinical and biological approach to risk stratification and treatment. Cell and Tissue Research. 372(2):195-209. doi:10.1007/s00441-018-2821-2.
13 ASCO. Neuroblastoma - Childhood: Stages and Groups. Available at: https://www.cancer.net/cancer-types/neuroblastoma-childhood/stages-and-groups