Neuroblastoma
🧒 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
