Prognosis

Overall: (1,2)

• stage I - 95% survival at 5 years
• stage IV - 20% survival at 5 years

Favourable prognosis associated with:

• age less than 1 year at diagnosis:
  • 50% 5 year survival for those diagnosed at 1 year
  • 20% 5 year survival for those diagnosed at 10 years
• stage I and II disease
• cervical, pelvic, mediastinal, orbital primary tumours
• normal neurone-specific enolase and ferritin at diagnosis
• high vanylmandelic to homovanillic acid ratio
• less than 3 copies of N-myc oncogene
• low telomerase activity
• stage IVS has a prognosis as good as stage II

Note

Modern treatment has improved outcomes, and 3-year survival rates now exceed 60%. (3) However, prognosis varies enormously, ranging from cure in more than 90% in low risk disease to below 50% for those with high risk disease. (4)

Clinical outcomes remain poor in patients with high-risk neuroblastoma, where chemo-resistant relapse is common following high-intensity conventional treatment. (5)

Reference

1. Irwin MS, Naranjo A, Zhang FF, et al. Revised neuroblastoma risk classification system: a report from the Children's Oncology Group. J Clin Oncol. 2021 Oct 10;39(29):3229-41.
2. Moreno L, Rubie H, Varo A, et al. Outcome of children with relapsed or refractory neuroblastoma: a meta-analysis of ITCC/SIOPEN European phase II clinical trials. Pediatr Blood Cancer. 2017 Jan;64(1):25-31.
3. Chung C, Boterberg T, Lucas J, et al; Neuroblastoma. Pediatr Blood Cancer. 2021 May;68 Suppl 2
4. Whittle SB, Smith V, Doherty E, et al; Overview and recent advances in the treatment of neuroblastoma. Expert Rev Anticancer Ther. 2017 Apr;17(4):369-386.
5. Barone G, Anderson J, Pearson AD, et al; New strategies in neuroblastoma: Therapeutic targeting of MYCN and ALK. Clin Cancer Res. 2013 Nov 1;19(21):5814-21