Prognosis

Prognosis depends primarily on the patient's risk classification.

Low-risk disease

Patients with low-risk disease have an excellent prognosis. Estimated 5-year overall survival in this population is 98%; 5-year event-free survival is 91%.[58]

Intermediate-risk disease

With a combination of surgery and chemotherapy, 5-year overall-survival and event-free survival is approximately 96% and 85%, respectively, in patients with intermediate-risk disease.[58]

High-risk disease

Despite intense multimodal therapy, patients with high-risk disease have a poor prognosis with an event-free survival of ≤50%.[58]

Relapsed and refractory disease

Survival rates for those with relapsed/refractory high-risk neuroblastoma remain poor. Meta-analysis of European phase 2 clinical trial data reported median overall survival of 16 months among children and adolescents with relapsed/refractory neuroblastoma.[115]​ 

One-year and 4-year overall survival rates were 57% and 20%, respectively, in a large cohort of patients with recurrent/refractory neuroblastoma from Children's Oncology Group (COG) modern-era early-phase trials.[116] Median time from diagnosis to first disease recurrence/progression was 18.7 months.

Long-term sequelae

Patients with low-risk disease who are observed or treated with surgery have minimal long-term sequelae. Because patients with intermediate- and high-risk disease are treated with chemotherapy and radiation in addition to surgery, survivors are at risk of experiencing treatment-related adverse effects (e.g., ototoxicity, cardiotoxicity, endocrine complications, osteoporosis, secondary malignancies, and future infertility), which may have long-term implications.[61][117]

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