Presents with palpitations, diaphoresis, pallor, and paroxysmal hypertension.
Risk factors include multiple endocrine neoplasia type 2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1.
Diagnosed by increased levels of urine and serum catecholamines, metanephrines, and normetanephrines.
Treatment includes medical options (phenoxybenzamine, phentolamine, alpha-blockers) and surgical options (open or laparoscopic adrenalectomy).
Complications include hypertensive crisis, myocardial infarction, and hypotension.
A tumor arising from catecholamine-producing chromaffin cells of the adrenal medulla that classically presents with headaches, diaphoresis, and palpitations in the setting of paroxysmal hypertension. Symptoms are usually episodic and tend to progress as the tumor grows. The majority of these tumors are benign. About 80% to 90% arise in the adrenal gland; the remainder being extra-adrenal in origin and most commonly found in the head and neck. These tumors mostly develop sporadically. Up to 35% of cases are a manifestation of a hereditary syndrome such as multiple endocrine neoplasia type 2 or Von Hippel-Lindau syndrome.     The tumor was named pheochromocytoma, after its characteristic "dusky-colored tumor" appearance, by Pick in 1912.
Associate Professor of Medicine
Medical College of Georgia
BS declares that she has no competing interests.
Dr Bridget Sinnott would like to gratefully acknowledge Dr Sidhbh Gallagher, the previous contributor to this monograph.
SG declares that she has no competing interests.
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