Neuroblastic tumors

Neuroblastic tumors arise from primitive cells of the sympathetic system and include the following entities:

These entities represent a spectrum of disease from undifferentiated and aggressive (neuroblastoma) to the well differentiated and largely quiescent (ganglioneuroma).

Epidemiology

The less differentiated, the earlier the presentation. Thus, neuroblastomas typically occur in infants and very young children (mean age of presentation 22 months) whereas ganglioneuroblastomas and ganglioneuromas present in older children (mean age of presentation 1-10 years and 6-15 years respectively) .

Pathology

As these tumors arise from the sympathetic nervous system, it is not surprising that they arise from the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis .

Treatment and prognosis

Prognosis matches degree of differentiation:

  • neuroblastoma
    • stage 1, 2 or 4S: 75-90% 3 year survival
    • stage 3
      • <1 year of age: 80-90% 1 year event free survival
      • >1 year of age: 50% 3 year survival
    • stage 4
      • <1 year of age: 60-75% 1 year event free survival
      • >1 year of age: 15% 3 year survival
  • ganglioneuroblastoma
    • intermediate prognosis
  • ganglioneuroma
    • complete resection is curative
    • no metastatic potential
    • local recurrence reported