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无功能性促肾上腺皮质腺瘤。

Silent corticotroph adenomas.

机构信息

Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Cedars-Sinai Medical Center, 127 S. San Vicente Blvd, A6600, Los Angeles, CA, 90048, USA.

出版信息

Pituitary. 2018 Apr;21(2):183-193. doi: 10.1007/s11102-018-0864-8.

Abstract

PURPOSE

Silent corticotroph adenomas (SCAs) present clinically as non-functioning adenomas (NFAs) but are immunopositive for adrenocorticotrophic hormone (ACTH) without biochemical and clinical manifestation of hypercortisolism. Pathologic examination of resected NFAs that demonstrate positive ACTH and/or TPIT expression confirms its corticotroph lineage. SCAs comprise up to 20% of NFAs and exhibit a higher rate of recurrence. Studies of molecular mechanisms have generated multiple hypotheses on SCA tumorigenesis, pathophysiology, and growth that as yet remain to be proven. An improved understanding of their pathologic and clinical characteristics is needed.

METHODS

A literature review was performed using PubMed to identify research reports and clinical case series on SCAs.

RESULTS

Up to date findings regarding epidemiology, mechanisms of pathogenesis, differentiation, progression, and growth, as well as clinical presentation, postoperative course, and treatment options for patients with SCAs are presented. Pooled results demonstrate that 25-40% of cases show cavernous sinus invasion, preoperative hypopituitarism, new-onset hypopituitarism, and recurrence.

CONCLUSION

This article reviews the incidence, molecular pathology, and clinical behavior of these unique non-functioning pituitary corticotroph adenomas, and highlights the need for rigorous monitoring for recurrences and hypopituitarism in patients with SCAs.

摘要

目的

静默促肾上腺皮质激素腺瘤(SCAs)在临床上表现为无功能腺瘤(NFAs),但免疫组化呈促肾上腺皮质激素(ACTH)阳性,无皮质醇增多症的生化和临床表现。对表现为 ACTH 和/或 TPIT 阳性表达的切除 NFA 的病理检查证实其为促皮质激素谱系。SCA 占 NFA 的 20%,复发率更高。对分子机制的研究提出了多种关于 SCA 肿瘤发生、病理生理学和生长的假说,但尚未得到证实。需要进一步了解其病理和临床特征。

方法

使用 PubMed 进行文献回顾,以确定关于 SCA 的研究报告和临床病例系列。

结果

目前的研究结果表明,SCA 的流行病学、发病机制、分化、进展和生长机制,以及临床表现、术后过程和治疗选择。汇总结果表明,25-40%的病例存在海绵窦侵犯、术前垂体功能减退、新发垂体功能减退和复发。

结论

本文综述了这些独特的无功能垂体促皮质激素腺瘤的发病率、分子病理学和临床行为,并强调了对 SCA 患者复发和垂体功能减退进行严格监测的必要性。

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