Makindo Medical Notes

🧒 Neuroblastoma is the most common solid tumour in children outside the brain, accounting for around 8% of childhood cancers. 📊 Fewer than 100 children are diagnosed each year in the UK.

🧬 About

• Embryonal tumour of neural crest origin.
• Part of a spectrum ranging from benign ganglioneuroma to highly malignant disease.

📍 Anatomy

• Most commonly arises in the adrenal medulla or sympathetic ganglia.
• Can occur anywhere along the sympathetic chain from neck to pelvis.
• Large tumours may cross the midline or encase great vessels.

🩺 Clinical Features

• 👶 Usually presents before 5 years old (median age ~18 months).
• Systemic: poor appetite, weight loss, lethargy.
• GI/Urinary: constipation, difficulty passing urine.
• Specific signs: periorbital bruising ("raccoon eyes"), breathlessness, leg weakness due to cord compression.
• Paraneoplastic syndromes (rare): opsoclonus-myoclonus ("dancing eyes–dancing feet").

🧪 Investigations

• 🧴 Urine catecholamine metabolites: ↑ vanillylmandelic acid (VMA) & homovanillic acid (HVA).
• 📸 Imaging: Chest X-ray, Ultrasound, CT/MRI for tumour size, spread, & spinal involvement.
• 🔬 Biopsy: histological confirmation.
• ☢️ MIBG scan: detects bone, marrow, and nodal spread.
• Bone marrow aspirate & trephine for staging.
• Bone scan for osseous involvement.

⚠️ Poor Prognostic Factors

• 🧬 N-myc amplification.
• Chromosome abnormalities: deletion of Chr 1p, gain of Chr 17q.
• Age > 1 year at diagnosis.
• Advanced stage, large size, metastatic disease.

📊 Staging (INSS)

• Stage I: Localised, completely resected (± microscopic residual).
• Stage IIA: Localised, incompletely resected, no nodes.
• Stage IIB: Localised with ipsilateral lymph nodes.
• Stage III: Tumour crosses midline ± contralateral nodes, unresectable.
• Stage IV: Distant spread (nodes, marrow, bone, liver, other organs).
• Stage IVS: Special infant stage (<1 yr) – localised tumour with limited spread to liver, skin, or marrow.

🛠️ Management

• Depends on age, stage, size, site, and biology.
• 🔪 Surgery: Stage I–II → resection ± adjuvant chemo.
• 💊 Chemotherapy: Induction therapy for advanced cases; high-dose chemo + stem cell transplant in high-risk disease.
• 🎯 Immunotherapy: Anti-GD2 monoclonal antibody + retinoic acid (differentiation therapy).
• ☢️ Radiotherapy: Post-surgery or post-high-dose chemo for residual disease.

📚 References

• Cancer Research UK: Neuroblastoma
• NHS: Neuroblastoma