SN6
SHORT NOTE
5M
Define Neuroblastoma. Describe its clinical features, investigations and prognosis.
Definition
- Neuroblastoma is an embryonal malignancy arising from neural crest cells of the sympathetic nervous system (adrenal medulla or sympathetic chain).
- It is the commonest extracranial solid tumour of childhood and usually occurs in children under 5 years.
Clinical Features
- An abdominal mass that is often irregular and crosses the midline (contrast with Wilms tumour); commonly arises from the adrenal.
- Features of metastatic disease — bone pain, periorbital bruising ('raccoon eyes') and proptosis, bone-marrow involvement, hepatomegaly.
- Catecholamine effects (hypertension, sweating, flushing) and paraneoplastic opsoclonus-myoclonus ('dancing eyes').
Investigations & Management
- Raised urinary catecholamine metabolites (VMA and HVA).
- Imaging (USG/CT/MRI), MIBG scan, and tissue biopsy (with MYCN amplification status for risk).
- Risk-based treatment — surgery, chemotherapy and radiotherapy; prognosis varies widely (infants often do well, and some tumours spontaneously regress, while high-risk disease has a poorer outcome).
Wilms Tumour VS Neuroblastoma
| Feature | Neuroblastoma | Wilms tumour |
|---|---|---|
| Origin | Sympathetic chain/adrenal | Kidney |
| Mass | Irregular, crosses midline | Smooth, doesn't cross midline |
| Markers | Urinary VMA/HVA | — |
| Child | Often ill, metastatic | Usually well |
Prognosis
Outcome depends on age, stage and biology (MYCN amplification) — infants and localised tumours do well (some regress spontaneously), whereas high-risk metastatic disease in older children has a poorer prognosis.
Staging & Special Features
- Staged by the extent of disease and resectability, and risk-grouped using age and MYCN status.
- Stage 4S (special, in infants) — disseminated but with a good prognosis and a tendency to spontaneous regression.
Biological / Prognostic Markers
- MYCN amplification — the key adverse prognostic marker (high-risk disease).
- Age (< 18 months favourable), stage, histology (Shimada) and DNA ploidy also stratify risk.
- Raised urinary VMA/HVA and serum ferritin/LDH/NSE support diagnosis and monitoring.
Crosses the midline — unlike Wilms tumour.
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