Epidemiology
- Most common malignancy of the adrenal medulla in infants
- Mean age at diagnosis: 1–2 years
Etiology
- Amplification and overexpression of oncogene MYCN (N-myc)
Pathophysiology
- Homer Wright rosettes: Halo-like clusters of neuroblast cells surrounding a central pale area containing neuropil (associated with tumors of neuronal origin such as neuroblastoma, medulloblastoma, primitive neuroectodermal tumors, and pineoblastoma)
- Bombesin and NSE positive
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A tumor marker for neuroblastoma, small cell carcinoma of the lung, pancreatic cancer, and gastric cancer.
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Clinical features
General symptoms
- Failure to thrive or weight loss
- Fever
- Nausea, vomiting, loss of appetite
Localized symptoms
Paraneoplastic syndromes
- Chronic diarrhea → electrolyte imbalances
- Opsoclonus-myoclonus-ataxia: a paraneoplastic syndrome of unclear etiology characterized by rapid and multi-directional eye movements, rhythmic jerks of the limbs, and ataxia (dancing eyes dancing feet syndrome)
- Possibly hypertension, tachycardia, palpitations, sweating, flushing (hypertension is more commonly seen in pheochromocytoma)
Diagnostics
Laboratory tests
- Urine
- ↑ Catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) in 24-hour urine
- Blood
- ↑ Catecholamine metabolites (HVA/VMA)
- ↑ Lactate dehydrogenase (LDH), ferritin, neuron-specific enolase (NSE)