Neuroblastoma

• What is Neuroblastoma
• Statistics on Neuroblastoma
• Risk Factors for Neuroblastoma
• Progression of Neuroblastoma
• Symptoms of Neuroblastoma
• Clinical Examination of Neuroblastoma
• How is Neuroblastoma Diagnosed?
• Prognosis of Neuroblastoma
• How is Neuroblastoma Treated?
• Neuroblastoma References

What is Neuroblastoma

Neuroblastoma is a cancer of childhood that grows in the nervous system.

Statistics on Neuroblastoma

Neuroblastoma is one of the most common childhood solid tumours and the most common tumour diagnosed in infants less than 1 year of age. About 80% of all neuroblastomas occur in children younger than 5 years. It accounts for at least 15% of all childhood cancer deaths. 500 new cases are diagnosed each year in the United States.

Risk Factors for Neuroblastoma

Most cases of neuroblastoma are without risk factors or known cause. In a minority of cases, neuroblastoma is passed on through families.

Progression of Neuroblastoma

The most important prognostic factors are the histological staging of the tumour and the age of the patient at diagnosis of neuroblastoma. Stage I and II tumours tend to occur in children younger than 1 year of age. Treatment in these instances has a 90% cure rate. Prognosis for stage III and IV tumours is less favourable, with cure rates of 20-30%. Metastases when they develop, do so early and widely. This is due to spread via the bloodstream in addition to the lymphatic and direct spread. There are different clinicobiologic subsets ranging from those that rarely kill to those that despite all therapeutic efforts, rapidly cause death.

How is Neuroblastoma Diagnosed?

Abdominal ultrasound– evaluation of an abdominal mass.

Prognosis of Neuroblastoma

Infants younger than one year of age have an excellent prognosis regardless of the stage of the neoplasm (95-98% 5 yr survival with full treatment). When limited to the liver, bone marrow and spleen, there is a greater than 80% 5 year survivial with just minimal therapy. When more widespread, the 5 year survival is still greater than 50% with minimal therapy. Children older than 1 year with stages 3-4 have a 10% survival rate despite all forms of treatment.

A staging system for the neuroblastoma tumour is as follows:

 

• Stage 1 – confined to organ of origin.
• Stage 2 – tumour extends in continuity beyond organ but does not cross the midline (+/- ipsilateral lymph nodes).
• Stage 3 – tumour extends in continuity beyond midline (+/- ipsilateral lymph nodes)
• Stage 4 – metastatic disease to viscera, distant lymph nodes, soft tissue and skeleton.
• Stage 4 – small adrenal tumours and extensive disease infiltrating liver, skin or bone marrow.
  

  How is Neuroblastoma Treated?

  Treatment of neuroblastoma is determined by the staging of the disease.

   

• Stage I and II tumours (local disease) tend to be treated with surgery.
• Stage III and IV tumours (extensive disease) are treated with chemotherapy to shrink the tumour mass and then surgery/radiotherapy if possible.
  

  Neuroblastoma References

  1. Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison’s Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001
  2. Cotran, Kumar, Collins 6th edition. Robbins Pathologic Basis of Disease. WB Saunders Company. 1999.
  3. Kumar P, Clark M. CLINICAL MEDICINE. WB Saunders 2002 Pg 427-430.
     Hurst JW (Editor-in-chief). Medicine for the practicing physician. 4th edition Appleton and Lange 1996.
  4. Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001