d'Herbomez Michèle, Rouaix Nathalie, Bauters Catherine, Wémeau Jean-Louis
Université de Lille II, F-59000 Lille, France.
Presse Med. 2009 Jun;38(6):927-34. doi: 10.1016/j.lpm.2008.08.011. Epub 2009 Jan 9.
Pheochromocytomas and/or paragangliomas are rare, heterogeneous tumors of the chromaffin cells. Thirty percent of the patients presented with these diseases in a hereditary context. The biological diagnosis relies on the identification of excessive secretion of the metanephrines which are more sensitive and specific than those of catecholamines The published recommendations give the opportunity to choose between the free metanephrines and the fractionated metanephrines in sera or urines. The concentrations of the free plasmatic metanephrines reflect the ongoing production of tumor. They are little sensitive to the renal failure. The assay of the vanillylmandelic acid should be dropped because of its inefficiency. The assay of the chromogranin A in serum should be used in association with those of metanephrines in the diagnosis but also in the follow-up. Its role still has to be precised.
嗜铬细胞瘤和/或副神经节瘤是罕见的、异质性的嗜铬细胞肿瘤。30%的患者在遗传背景下患有这些疾病。生物学诊断依赖于鉴定甲氧基肾上腺素的过度分泌,其比儿茶酚胺更敏感和特异。已发表的建议提供了在血清或尿液中选择游离甲氧基肾上腺素和分馏甲氧基肾上腺素的机会。游离血浆甲氧基肾上腺素的浓度反映了肿瘤的持续产生。它们对肾衰竭不太敏感。由于香草扁桃酸检测效率低下,应放弃该检测。血清嗜铬粒蛋白A检测应与甲氧基肾上腺素检测联合用于诊断和随访。其作用仍有待明确。