Neuroblastoma

Malignant neuroendocrine tumour of the sympathetic nervous system that originates from neural crest cells

Aetiology

  • Affects 1 in 7 000 live births
  • Usually diagnosed 18 months, 40% diagnosed in infancy
  • 40% arise in the adrenal medulla, remainder mostly occur along the sympathetic chain
  • Composed of primative appearing cells but can show maturation and differentiation towards gangliod cells
  • Age and stage important for prognosis
  • Amplification of N-myc and expression of telomerase predict a poor outcome

Clinical presentation

  • Usually presents quite late - majority of symptoms are due to mass effects of the tumour or as a result of metastasis
  • Symptoms include:
    • Loss of appetite
    • Vomiting
    • Weight loss
    • Fatigue
    • Bone pain

Investigations

  • Bloods - FBC may detect anaemia, raised inflammatory markers, abnormal coagulation tests
  • CT
  • Biopsy

Management

Depends on risk stratification:
  • Low risk patients are simply observed for spontaneous resolution or are treated with local resection
  • HIgh risk patients are given multi-agent chemotherapy, surgery and radiotherapy