良性肾上腺皮质肿瘤中的两性异形现象。

IF 5.3 1区 医学 Q1 ENDOCRINOLOGY & METABOLISM
Onnicha Suntornlohanakul, Cristina L Ronchi, Wiebke Arlt, Alessandro Prete
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引用次数: 0

摘要

良性肾上腺皮质肿瘤是最常见的肾上腺肿瘤。过去几十年的证据强调了其患病率、临床特征和治疗结果的性别差异。产生皮质醇的腺瘤引起库欣综合征,特别是那些PRKACA或GNAS体细胞突变与更严重的表型相关的库欣综合征,或轻度自主皮质醇分泌(MACS)在女性中更常见。支持这种两性二态性的机制仍有待充分阐明。对小鼠的研究揭示了雄激素对雄性的保护作用,导致肾上腺皮质细胞的生长速度减慢。此外,来自人类肾上腺肿瘤组织的证据表明,肾上腺皮质中的雌激素、黄体酮和促黄体生成素/绒毛膜促性腺激素信号传导可能在肾上腺肿瘤的发生和类固醇的产生中发挥作用。在临床上,在青春期、妊娠期和绝经期产生皮质醇的肾上腺皮质腺瘤或结节性增生的发生率增加支持了这一点。值得注意的是,患有MACS的女性似乎更容易受到皮质醇过量的有害影响,死亡风险也高于男性。患有醛固酮产生性腺瘤的女性比男性有更高的体细胞KCNJ5突变的患病率,携带这些突变的患者在肾上腺切除术后可能有更有利的临床结果。在这篇综述中,我们总结了良性肾上腺皮质肿瘤性别二型性背后的可能机制,并提供了其患病率、临床表现和结局的性别特异性差异的最新概述,重点是皮质醇和醛固酮过量。考虑两性异形是至关重要的指导诊断和管理,并建议这些患者的最佳护理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sexual dimorphism in benign adrenocortical tumours.

Benign adrenocortical tumours are the most common adrenal neoplasms. Evidence over the past few decades has highlighted sex differences in their prevalence, clinical characteristics, and treatment outcomes. Cortisol-producing adenomas causing either Cushing's syndrome, particularly those with PRKACA or GNAS somatic mutations associated with a more severe phenotype, or mild autonomous cortisol secretion (MACS) are more commonly observed in women. The mechanisms underpinning this sexual dimorphism remain to be fully elucidated. Studies in mice have revealed a protective role of androgens in males, leading to a decelerated growth rate of adrenocortical cells. Furthermore, evidence from human adrenal tumour tissue suggests that oestrogen, progesterone, and luteinising hormone/choriogonadotropin signalling in the adrenal cortex may play a role in adrenal tumourigenesis and steroid production. Clinically, this is supported by the increased incidence of cortisol-producing adrenocortical adenomas or nodular hyperplasia during puberty, pregnancy, and menopause. Notably, women with MACS seem to be more vulnerable to the harmful effects of cortisol excess and carry a higher mortality risk than men. Women with aldosterone-producing adenomas have a higher prevalence of somatic KCNJ5 mutations than men, and patients harbouring these mutations are likely to have more favourable clinical outcomes after adrenalectomy. In this review, we summarise the possible mechanisms behind the sexual dimorphism of benign adrenocortical tumours and provide an up-to-date overview of the sex-specific differences in their prevalence, clinical presentation, and outcomes, focusing on cortisol and aldosterone excess. Considering sexual dimorphism is crucial to guide diagnosis and management, and to counsel these patients for optimised care.

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来源期刊
European Journal of Endocrinology
European Journal of Endocrinology 医学-内分泌学与代谢
CiteScore
9.80
自引率
3.40%
发文量
354
审稿时长
1 months
期刊介绍: European Journal of Endocrinology is the official journal of the European Society of Endocrinology. Its predecessor journal is Acta Endocrinologica. The journal publishes high-quality original clinical and translational research papers and reviews in paediatric and adult endocrinology, as well as clinical practice guidelines, position statements and debates. Case reports will only be considered if they represent exceptional insights or advances in clinical endocrinology. Topics covered include, but are not limited to, Adrenal and Steroid, Bone and Mineral Metabolism, Hormones and Cancer, Pituitary and Hypothalamus, Thyroid and Reproduction. In the field of Diabetes, Obesity and Metabolism we welcome manuscripts addressing endocrine mechanisms of disease and its complications, management of obesity/diabetes in the context of other endocrine conditions, or aspects of complex disease management. Reports may encompass natural history studies, mechanistic studies, or clinical trials. Equal consideration is given to all manuscripts in English from any country.
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