Endocrine Abstracts

Pheochromocytomas (PH) and functional paragangliomas (PGL) are neoplasms of chromaffin tissue that synthesize catecholamines. The diagnosis of PH or functional PGL is based on the determination of metanephrines. Most catecholamine-secreting tumors arise in the adrenal glands (PH proper) and are easily detected by computed tomography or magnetic resonance imaging. Patients may also harbor extraadrenal primary tumors (PGL) or distant metastases. Adrenal imaging should therefore be combined with whole-body metaiodobenzylguanidine scintigraphy. One in four patients with PH or PGL has germline mutations conferring a predisposition to catecholamine-secreting tumors. The identification of a causative mutation should lead to presymptomatic genetic testing in the family. Patients with catecholamine-secreting tumors should be referred to centers with extensive experience in the anesthetic and surgical management of the disease. Blood pressure should be normalized before surgery, using alpha-adrenergic and possibly beta-adrenergic antagonists. Most PH and many PGL can be resected laparoscopically. Adrenal cortex-sparing surgery is feasible in patients with bilateral PH. PH and PGL may recur. Patients should be subject to life-long follow-up, with check-ups at least one yearly including blood pressure measurement and metanephrine determination