Sporadic Noradrenergic Adrenal Pheochromocytoma in an Adolescent Patient

Pheochromocytoma and paraganglioma are neuroendocrine tumors that occur less commonly among children compared to adults. The excess catecholamines secreted by the tumor cells result in hypertension, tachycardia, excess sweating, and headache. Other symptoms include abdominal pain or distension caused by the adrenal mass. Here, we report a case of pheochromocytoma arising from the left adrenal medulla in a 14-year-old boy, which was exclusively secreting norepinephrine, as suggested by elevated plasma and 24-hour urinary norepinephrine and its metabolite normetanephrine. The epinephrine and its metabolite metanephrine were within normal limits. He presented with abdominal pain, recurrent vomiting, and headache and was noted to have elevated blood pressure. He underwent adrenalectomy after controlling his blood pressure with an alpha-blocker Prazosin. His blood pressure remained stable after surgery, and his plasma-free metanephrines returned to normal limits. He tested negative for hereditary paraganglioma-pheochromocytoma gene panel.