Most common tumor of the adrenal medulla in children, usually <4 years old. Originates from neural crest cells. Occurs anywhere along the sympathetic chain.

Most common presentation:- Abdominal distension - Firm, irregular mass that can cross the midline (vs Wilms tumor, which is smooth and unilateral)- Less likely to develop hypertension than with pheochromocytoma- Can present with opsoclonus-myoclonus syndrome ("dancing eyes-dancing feet")

- ↑ homovallinic acid (HVA) and vanillylmandelic acid (VMA) in urine (catecholamine metabolites).- Homer-Wright rosettes characteristic of neuroblastoma and medulloblastoma- Bombesin and NSE ⊕- Associated with overexpression of N-myc oncogene- Classified as an APUD tumor