Neuroblastoma

 

Neuroblastoma is a common childhood neoplasm and is the most common extracranial solid tumor in children.

Epidemiology

  • Incidence – 10.5/1,000,000 children <15 years old worldwide
  • Age – median is 2 years
  • Sex – slightly higher female predominance
  • Occurrence – mostly sporadic, 1-2% are familial

Risk Factors 

  • Neurofibromatosis
  • Type I congenital central hypoventilation syndrome
  • Hirschsprung disease

Pathophysiology

  • Malignant tumor consisting of poorly differentiated neuroectodermal cells derived from the neural crest

Clinical Presentation

  • Determined by tumor location and stage
  • Localized tumors are often asymptomatic (25-40% of patients)
  • Metastatic tumors frequently associated with fever, bone pain and weight loss
    • Orbital metastases – ecchymoses called raccoon eyes
    • Paraspinal disease – paresis and cord compression
    • Cervical, apical thoracic disease – Horner syndrome
  • Syndromes associated with neuroblastoma
    • Pepper syndrome – massive involvement of liver with metastatic disease
    • Horner syndrome – ptosis, miosis and anhidrosis
    • Hutchinson syndrome – limping and irritability associated with bone and bone marrow metastases
    • Paraneoplastic syndromes
      • Opsoclonus-myoclonus syndrome (dancing eyes, dancing feet)
        • Involuntary eye fluttering
        • Muscle jerking
        • Ataxia
      • Vasoactive intestinal polypeptide – refractory diarrhea, failure to thrive
    • Neurocristopathy syndrome – neuroblastoma associated with other neural crest disorders