Pheochromocytoma is a neuroendocrine tumor of the adrenal glands that can release high levels of epinephrine and norepinephrine. The signs and symptoms are palpitations, increased heart rate, increased BP, weight loss, diaphoresis etc. It can also cause resistant arterial hypertension, be fatal if it causes a hypertensive emergency. Up to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1, SDHB and SDHD are all known to cause familial pheochromocytoma. It is a tumor of the multiple endocrine neoplasia syndrome, type IIA and type IIB. The incidence is 2 to 8 per million persons per year, present in 0.1% to 1% of patients with hypertension and it is present in approximately 5% of patients with incidentally discovered adrenal masses. The peak incidence occurs in the third to fifth decades of life and is equal between males and females.
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