Neuroblastoma is a common cancer of infancy and childhood.
It presents with a mass in the abdomen and frequently evidence of spread to other parts of the body.
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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.
What is 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, lymphoma and brain tumours) and is responsible for 15% of the 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 of neuroblastoma
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.
How is neuroblastoma diagnosed?
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.
How is neuroblastoma treated and what is the outcome?
Children under 12 months and all children with 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.
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