Abstract

COSTA, Liane  and  GOMES, António Taveira. Pheochromocytoma. Arq Med [online]. 2008, vol.22, n.6, pp.177-187. ISSN 2183-2447.

Pheochromocytoma is a rare, catecholamine-producing tumour, usually arising from the adrenal medulla. It has a highly variable clinical presentation but headaches, palpitations, sweating and arterial hypertension are very characteristic. The paroxysmal hypertensive crisis and the potentially fatal cardiovascular events that can occur are due to the powerful effect of the catecholamine stimulation. Therefore, a prompt diagnosis and therapeutical approach are strongly justified, in order to assure the rapid control of the symptoms and the prevention of complications. The diagnosis of pheochromocytoma lies in the biochemical identification of excessive production of catecholamines or their metabolites, either in blood or urine. These biochemical tests should be performed in every patient with suggestive signs or symptoms, in the asymptomatic patients with an adrenal incidentaloma and in those with recognized genetic syndromes which predispose to the disease (multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1 syndrome, paraganglioma syndromes, etc.). The imaging techniques used to locate biochemically proven tumours are CT (computerized tomography), MRI (magnetic resonance imaging) and techniques with functional ligands, such as scintigraphy with MIBG - I¹³¹ (radioactive Iodine metaidobenzoguanidine). The definitive treatment for any pheochromocytoma, especially if it is functional and symptomatic, is surgical. Laparoscopic adrenalectomy is now the preferred surgical technique for the removal of pheochromocytoma. Pheochromocytoma has a great potential for cure, after early diagnosis and treatment, but it is much poorer when there is evidence of tumoral invasion or metastases.

Keywords : pheochromocytoma; paraganglioma; catecholamines; metanephrines; arterialhypertension; laparoscopic adrenalectomy.

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