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Neuroblastoma

Neuroblastoma is a disease that arises from the neural crest tissue of the adrenal gland and sympathetic nervous tissue. It occurs most often in children under the age of five. Commonly, the primary is of the adrenal gland (located above the kidneys). Most children present with an abdominal mass. This mass is often large and complex, crossing the midline and incorporating major blood vessels and lymph glands.

Signs and Symptoms

The symptoms of neuroblastoma are often vague and thus early-stage diagnosis can be rare. This is why neuroblastomas diagnosed in children over two years of age have often already metastasised (spread) at presentation, causing some or all of the following presentational signs:

  • Pallor (pale appearance of the skin)
  • Weight loss
  • Hepatomegaly (enlargement of the liver)
  • Bone pain and Limp
  • Proptosis (forwardly displaced or ‘bulging’ eye)
  • Periorbital bruising (‘black’ or ‘raccoon’ eye)
  • Skin nodules.

Spinal cord compression (pressure on the spinal cord) may also be a clinical feature if there is a para-vertebral tumour (tumour around the spine).

Diagnosis

The diagnosis is established on biopsy, which may be through a needle, or on operation. Once it is diagnosed staging work up with bone scan and bone marrow is essential to find whether the disease is localized or had spread. A specific scan may be used to assess the extent of the disease (for example, an mIBG scan, involving the injection of metaiodobenzyl guanidine which is taken up by neuroblastoma cells and is highlighted by radiation scanning).

Treatment

The size, position and stage of the tumour, and the age of the child, dictate the treatment offered. Localized diseases are treated with surgery alone with or without chemotherapy. Advanced disease needs intensive chemotherapy followed by surgery and or radiotherapy. In stage IV disease treatment involves high dose chemotherapy and bone marrow transplantation.

Prognosis

Unfortunately, the majority of children with neuroblastoma present with metastatic disease and the prognosis for this disease remains poor. Only 30% of children can expect a cure. However if disease is localised at diagnosis the prognosis is much improved. In young infants this disease has been seen to spontaneously regress without intervention, becoming benign.