Treatment

Treatment depends on staging. (1)

Observation is recommended for: (2)

• Low-risk MS disease that is asymptomatic with favourable biology; these patients have a high rate of spontaneous regression
• Infants <6 months with L1 disease with a small isolated adrenal mass (<5 cm diameter); tumour enlargement in these patients warrants surgery

Observation is accompanied by serial ultrasound (e.g., at 3-6 week intervals, or as clinically indicated), and should continue at increasing intervals over a 2-year period.

Surgical resection is recommended for all other patients with low-risk stage L1 disease:

• stage I - surgery alone
• stage II - surgery and local radiotherapy to tumour bed
• stage III - surgery, chemotherapy and radiotherapy
• stage IV - chemotherapy

Neuroblastomas are radiosensitive. Agents used in chemotherapy are varied and include carboplatin, etoposide, cyclophosphamide, and doxorubicin.

Note

High-risk disease can be difficult to cure, and a large proportion of these patients experience recurrence. Patients with high-risk disease are now therefore treated aggressively with multimodal therapy including chemotherapy, surgery, autologous bone marrow transplant, radiation, post-consolidation therapy (dinutuximab plus isotretinoin), and continuation therapy (eflornithine) (2)

Reference

1. Shohet J, Foster J; Neuroblastoma. BMJ. 2017 May 3;357
2. National Comprehensive Cancer Network. Neuroblastoma [internet publication].