Department of Endocrinology, Diabetes and Rheumatology Clinic of Nuclear Medicine Institute of Pathology, University Hospital Duesseldorf, Moorenstrasse 5, D-40225 Duesseldorf, Germany.
Eur J Endocrinol. 2011 Apr;164(4):447-55. doi: 10.1530/EJE-10-1070. Epub 2011 Jan 26.
Current guidelines suggest proving angiotensin-independent aldosterone secretion in patients with primary aldosteronism (PA). It is further recommended to demonstrate unilateral disease because of its consequence for therapy. A general screening for excess secretion of other hormones is not recommended. However, clinically relevant autonomous aldosterone production rarely originates in adrenal tumors, compromised of zona glomerulosa cells only. This article reviews published data on aldosterone- and cortisol-co-secreting tumors and shows that pre-operative diagnosis of such a lesion is beneficial for patients. Overt or subclinical glucocorticoid hypersecretion may interfere with diagnostic studies, e.g. adrenal venous sampling, screening of familial forms of PA on the basis of serum 18-hydroxy-cortisol (18-OH-F) determination, and provoke glucocorticoid deficiency after surgical removal of the tumor. In addition, knowledge from histological and molecular studies in patients with aldosterone- and cortisol-co-secreting tumors challenges some concepts of the development of adrenal autonomy. The presence of an aldosterone- and cortisol-co-secreting adrenocortical tumor should be considered if a patient has i) PA and an adenoma that is larger than 2.5 cm, ii) cortisol that is non-suppressible with overnight low-dose dexamethasone, or iii) grossly elevated serum levels of hybrid steroids, such as 18-OH-F.
目前的指南建议在原发性醛固酮增多症（PA）患者中证实血管紧张素非依赖性醛固酮分泌。进一步建议证明单侧疾病，因为这对治疗有影响。不建议对其他激素的过度分泌进行一般筛查。然而，临床上相关的自主醛固酮产生很少源自仅由球状带细胞组成的肾上腺肿瘤。本文回顾了关于醛固酮和皮质醇共同分泌肿瘤的已发表数据，并表明术前诊断此类病变对患者有益。显性或亚临床糖皮质激素分泌过多可能会干扰诊断研究，例如肾上腺静脉采样、基于血清 18-羟基皮质醇（18-OH-F）测定的 PA 家族形式的筛查，以及在肿瘤切除后引起糖皮质激素缺乏。此外，来自醛固酮和皮质醇共同分泌肿瘤患者的组织学和分子研究的知识挑战了肾上腺自主性发展的一些概念。如果患者存在以下情况，应考虑存在醛固酮和皮质醇共同分泌的肾上腺皮质肿瘤：i）PA 和大于 2.5cm 的腺瘤，ii）隔夜小剂量地塞米松不能抑制皮质醇，或 iii）混合类固醇（如 18-OH-F）的血清水平显著升高。
