Delèvaux I, Cabane J, Picard O, Bénit C, Lebas J, Nordlinger B, de Saint-Maur P, Imbert J C
Service de Médecine interne, Hôpital Saint-Antonie, Paris.
Presse Med. 1998 Sep 5;27(25):1272-4.
Pheochromocytoma and primary hyperaldosteronism rarely occur simultaneously. Few cases have been reported in the literature.
A patient explored for hypertension was found to have hypokalemia related to primary hyperaldosteronism. Pathology examination of the ablated adrenal showed a co-existing pheochromocytoma suspected at history taking although urine catecholamines were normal.
Different pathogenic hypothesis have been proposed. Such dual tumors could be a simple coincidence, occur in a particular genetic setting, be related to direct contact between cortical and medullary tissue leading to reactional cortical hyperplasia, pheochromocytoma produced factors stimulating aldosterone synthesis, or factor X, a substance produced by cortical adenomas and favoring growth of the pheochromocytoma.
嗜铬细胞瘤和原发性醛固酮增多症很少同时发生。文献中报道的病例较少。
一名因高血压接受检查的患者被发现患有与原发性醛固酮增多症相关的低钾血症。切除的肾上腺病理检查显示存在嗜铬细胞瘤,尽管尿儿茶酚胺正常,但在病史采集时就怀疑有这种并存情况。
已经提出了不同的致病假说。这种双肿瘤可能只是简单的巧合,发生在特定的基因背景下,与皮质和髓质组织之间的直接接触导致反应性皮质增生有关,嗜铬细胞瘤产生刺激醛固酮合成的因子,或者是因子X,一种由皮质腺瘤产生并有利于嗜铬细胞瘤生长的物质。