Álvarez TR, Álvarez TR, Portela OJM, Olvera BC, Burgos ZÁ

Language: Spanish
References: 18
Page: 148-156
PDF size: 227.02 Kb.

ABSTRACT

Pheochromocytomas are rare tumours of chromaffin cells embryologically arising from neural crest tissue. They are catecholamine-secreting tumours: noradrenalin, adrenaline and small quantities of dopamine. Their cells may produce and secrete a variety of neuropeptides and inflammatory cytokines that could affect tissues with its consequent signs and symptoms. The traditional rule of ten has not been modified; it may be bilateral in 10% of the cases, extra adrenal in 10% of the cases, family related in 10% of the cases and malignant in 10% of the cases. The bilateral pheocromocytoma prevalence is above 10% mainly among familiar syndrome like Von Hippel-Lindau Syndrome or MEN2. Extra adrenal tumours are close to 20% and about 6% of them are hereditary. Finally even though pheocromocytoma metastasis are rare, 5% of the cases will have metastasis at the moment of diagnosis and 33% of extra-adrenal cases will have them, including patients with familiar paragangliomas with specific mutations. Diagnosis is based on clinical findings compatible with paroxystic adrenaline liberation and confirmed by biochemical studies such as metanephrines, normetanephrines, catecholamines, vanilmandelic acid and «Y» neuropeptid in a 24 hour urine and serum sample. Computer multicut tomography (Figures 1 A y B) is the gold standard to diagnose pheochromocytoma, new diagnostic procedures such as PET scan with 18F-fluordopamine and 18F-fluorhidroxyphenilalanine to generate functional mapping and to localize metastases have become more popular.

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