An unusual and interesting case of phaeochromocytoma

Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla. The common age of onset is between the third and fifth decades of life. Commonly adrenal phaeochromocytoma secretes epinephrine (its metabolite metanephrine). Extra-adrenal phaeochromocytoma secretes norepinephrine and normetanephrine. Herein, we present the case of a 13-year-old male patient who presented with palpitations, giddiness and increased appetite for 3 days. He was found to have de novo hypertension. Imaging showed bilateral adrenal masses. Biochemical analysis revealed normal levels of plasma and 24-h urinary metanephrines. On further evaluation, serum metanephrine-fractionated assay showed marked elevation in normetanephrine levels. The patient was initially managed medically with alpha- and beta-blockers. After controlling blood pressure, adrenalectomy was done. The histopathological examination was suggestive of phaeochromocytoma. Adrenal phaeochromocytoma-secreting normetanephrines are a rare entity. On reviewing literature, only a few cases of this type were published.