Feocromocitoma

 

Liane Costa*, António Taveira Gomes*†

*Faculdade de Medicina da Universidade do Porto; †Serviço de Cirurgia Geral, Hospital de São João, Porto

 

O feocromocitoma é uma neoplasia rara, produtora de catecolaminas, cuja origem mais frequente é a medula su-pra-renal. Tem um amplo espectro de apresentações clínicas mas a tríade sintomática de cefaleias, palpitações e hipersudorese, acompanhada de hipertensão arterial é muito característica. As crises hipertensivas e os eventos cardiovasculares potencialmente fatais causados por esta neoplasia decorrem do efeito potente da estimulação adrenérgica e justificam a importância de um diagnóstico célere e de uma abordagem terapêutica eficaz no controlo sintomático e na prevenção de complicações. O diagnóstico de feocromocitoma é obtido por testes bioquímicos sanguíneos ou urinários em que se evidencie excesso de catecolaminas ou dos seus metabolitos. Estes devem ser realizados em todos os doentes com clínica sugestiva, nos indivíduos assintomáticos que apresentem um incidentaloma supra-renal ou naqueles em que exista predisposição genética conhecida (p.e. síndrome de neoplasias endócrinas múltiplas tipo 2, doença de von Hippel Lindau, neurofibromatose tipo 1, síndrome de paragangliomas familiares). A avaliação imagiológica de um feocromocitoma – através de TC (tomografia computorizada), RMN (ressonância magnética) ou estudos com ligandos funcionais, como a cintigrafia com MIBG-I¹³¹ (metaiodobenzilguanidina marcada com iodo radioactivo) – deve ser efectuada após confirmação diagnóstica e é muito útil na determinação da localização e dimensões tumorais. O tratamento definitivo de qualquer feocromocitoma, especialmente se funcionante e sintomático, é cirúrgico e, actualmente, consiste quase sempre na adrenalectomia total laparoscópica. O potencial de cura desta neoplasia, se diagnosticada e tratada atempadamente, é enorme mas, nos casos em que se constata invasão tumoral ou metastização, o prognóstico é bastante mais reservado.

Palavras-chave: feocromocitoma; paraganglioma; catecolaminas; metanefrinas; hipertensão arterial; adrenalectomia laparoscópica.

 

 

Pheochromocytoma

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.

Key-words: pheochromocytoma; paraganglioma; catecholamines; metanephrines; arterialhypertension;laparoscopic adrenalectomy.

 

 

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Correspondência:

Drª. Liane Costa

Faculdade de Medicina da Universidade do Porto

Alameda Prof. Hernâni Monteiro

4200-319 Porto

e-mail: liane@med.up.pt