Pheochromocytoma surgery

Overview
A pheochromocytoma (phaeochromocytoma outside of the US) is a neuroendocrine tumor of the medulla of the adrenal glands (originating in the chromaffin cells) or extra-adrenal chromaffin tissue which failed to involute after birth, which secretes excessive amounts of catecholamines, usually epinephrine and norepinephrine. Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin.

Surgery
Surgical resection of the tumor is the treatment of first choice. Given the complexity of perioperative management, and the potential for catastrophic intra and postoperative complications, such surgery should be performed only at centers experienced in the area. In addition to the surgical expertise that such centers can provide, they will also have the necessary endocrine and anesthesia resources as well. It may also be nescessary to carry out adrenalectomy, a complete surgical removal of the affected adrenal gland(s).

Either surgical option requires prior treatment with both the non-specific alpha adrenoceptor blocker Phenoxybenzamine to counteract hypertension and the beta-1 adrenoceptor antagonist Atenolol to reduce cardiac output. Given before surgery, these can also block the effect of a sudden release of adrenaline during tumour removal, which would otherwise endanger the anaethetised patient.

Phäochromozytom Feocromocitoma Phéochromocytome Feocromocitoma פאוכרומוציטומה Feochromocytoom 褐色細胞腫 Guz chromochłonny nadnerczy Feokromocytom