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11 March 2011

Neuroblastoma

Neuroblastoma is the most common cancer of infancy, with a median age of onset at 20 months. It is also the fourth commonest cancer in childhood (after leukaemia and lymphoma).

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Summary

  • Neuroblastoma is a common cancer of infancy and childhood.
  • It presents with a mass in the abdomen and frequently with evidence of spread to other parts of the body.
  • When the diagnosis is made early, surgery offers good results.
  • Once the tumour has spread, however, even with radiotherapy and chemotherapy the prognosis is poor.

Description

Neuroblastoma is the most common cancer of infancy, with a median age of onset at 20 months. It is also the fourth commonest cancer in childhood (after leukaemia, lymphoma and brain tumours) and is responsible for 15% of cancer deaths in childhood. These tumours start when cells in the adrenal gland or the cells of the sympathetic nervous system (often in ganglia next to the spine) divide in an uncontrolled, abnormal way.

Symptoms and signs 

The most common way for neuroblastoma to present is as a mass in the child’s abdomen that is noticed on examination following pain or poor feeding.

Sometimes an X-ray, done for another reason, will demonstrate deposits of calcium in the tumour.

Neuroblastoma can spread widely, and may have already done so at the time of first diagnosis. Such metastatic spread may cause chest complaints, skin lesions, neurological problems, a limp, back pain and associated fever and weight loss.

Diagnosis  

Diagnosis of neuroblastoma involves a variety of specialised tests, including urine analysis to measure chemical products of the tumour, CT, bone scans and bone marrow biopsy to identify its location and spread, and biopsy of the tissue of the tumour itself. This work-up also allows grading and staging of the tumour and an estimation of prognosis.

Treatment and outcome 

Children under 12 months, and all children with the disease that has not spread generally, have a better prognosis. Children who have localised disease may be cured by complete surgical removal of the tumour. When the tumour is more advanced, chemotherapy is the mainstay of treatment. It may render an inoperable tumour operable. The prognosis in advanced disease (especially if spread to the bones) may be as poor as 10-20% survival at five years, whereas with early, localised disease this is 90% or higher. Unfortunately, most patients have more advanced disease at diagnosis.

Reviewed by Prof P.S. Hartley, MBChB, FCPaed., Associate Professor and Head Paediatric Cancer unit, Red Cross Childrens Hospital.

Reviewed by Dr C Jacobs

Most recent review by Dr David Eedes, Oncologist, February 2011

 
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