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Neuroblastoma — Clinical Guidelines

Overview

Neuroblastoma is the most common extracranial solid tumor in children, arising from neural crest cells, primarily affecting the adrenal glands and sympathetic ganglia 1.

Diagnosis

Imaging studies (MRI, CT, MIBG scintigraphy) for tumor localization and staging 1 15 35.

Biopsy and histopathological examination for definitive diagnosis 14 44.

Elevated plasma renin levels may be observed 37.

Mass screening can detect early-stage disease, particularly in neonates 1 37.

Management

First-line treatments:

- Chemotherapy: Combination regimens such as induction therapy followed by consolidation (e.g., vincristine, doxorubicin, cyclophosphamide, and cisplatin) 1. - Anti-GD2 monoclonal antibodies: Dinutuximab in combination with isotretinoin, interleukin-2, and GM-CSF for high-risk patients 14 2. - Radiation therapy: Palliative use for symptomatic relief in metastatic disease 24 25.

Adjunctive therapies:

- Targeted radiotherapy: 131I-MIBG for refractory cases 9 25. - CAR T-cell therapy: Emerging as a promising treatment option for refractory neuroblastoma 6.

Special Populations

Pediatrics: Focus on minimizing toxicity and optimizing supportive care, including extended sedation protocols for MIBG therapy 13.

Comorbidities: Consider specific management adjustments for patients with additional health issues, such as those requiring palliative radiotherapy 24.

Key Recommendations

Use multimodal therapy including chemotherapy, anti-GD2 monoclonal antibodies, and potentially CAR T-cell therapy for high-risk neuroblastoma (Evidence: Strong 1 6).

Employ MIBG scintigraphy for accurate staging and monitoring response to treatment (Evidence: Moderate 35).

Implement careful sedation protocols during radiopharmaceutical therapy to ensure patient safety (Evidence: Moderate 13).

Consider targeted radiotherapy with 131I-MIBG for patients with refractory disease (Evidence: Moderate 9 25).

Monitor vitamin D status in high-risk neuroblastoma patients due to potential deficiency concerns (Evidence: Moderate 8).

References

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Neuroblastoma