Pheochromocytomas are rare disorders in children. It is a tumor that arises from the adrenal medulla and secretes excessive catecholamines. Intermittent hypertension is the classic presentation but sustained hypertension with or without paroxysms is also seen clinically. A common description of paroxysms refers to the “5 Ps” –pressure (sudden increase in blood pressure), pain (headache, chest, abdomen), perspiration, palpitations, and pallor. Children are more at risk than adults for malignant disease, and about 10% of diagnoses occur in the pediatric population. Children are also more likely to have recurrent disease especially in those with familial pheochromocytoma.

Appropriate screening should be performed when there is a high index of suspicion. Initial diagnosis is made by 24-hour urine collection and measurement of free catecholamines (norepinephrine and epinephrine) or their metabolites (vanillylmandelic acid [VMA] and total metanephrine). Plasma metanephrine measurements may also be useful in children who may provide an inaccurate 24-hour urine sample. Several imaging techniques, such as computed tomography, magnetic resonance imaging, or nuclear scan, can localize the lesion.