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解决双侧肾上腺结节性促肾上腺皮质激素非依赖性库欣综合征的定位临床难题。

Addressing the Clinical Conundrum of Localization in ACTH-independent Cushing Syndrome With Bilateral Adrenal Nodules.

作者信息

Chan Soo Ling, Chatterjea Rhea, Cheah Wei Keat, Oh Han Boon

机构信息

Department of Medicine, Ng Teng Fong General Hospital, Singapore 609606, Singapore.

Department of Surgery, Ng Teng Fong General Hospital, Singapore 609606, Singapore.

出版信息

JCEM Case Rep. 2025 Sep 24;3(10):luaf192. doi: 10.1210/jcemcr/luaf192. eCollection 2025 Oct.

DOI:10.1210/jcemcr/luaf192
PMID:41000299
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12457819/
Abstract

ACTH-independent Cushing syndrome is commonly caused by a unilateral adrenal adenoma. However, in cases with bilateral adrenal nodules, localization of the functional lesion is challenging. We present the case of a 26-year-old woman with ACTH-independent Cushing syndrome and concurrent bilateral adrenal nodules. Using dexamethasone-suppressed adrenal venous sampling (AVS), an adrenal vein to peripheral vein cortisol ratio >6.5, and an aldosterone-corrected cortisol lateralization ratio >2.3, the autonomous cortisol secretion was localized to the right adrenal adenoma. She underwent a right posterior retroperitoneal laparoscopic adrenalectomy resulting in clinical and biochemical resolution of hypercortisolism. Postoperatively, she required hydrocortisone therapy, which was successfully discontinued after 4 years. The use of dexamethasone-suppressed AVS, in conjunction with aldosterone-corrected cortisol lateralization ratio, may enhance the diagnostic accuracy of AVS.

摘要

促肾上腺皮质激素(ACTH)非依赖性库欣综合征通常由单侧肾上腺腺瘤引起。然而,在双侧肾上腺结节的病例中,功能性病变的定位具有挑战性。我们报告一例26岁患有ACTH非依赖性库欣综合征并伴有双侧肾上腺结节的女性病例。使用地塞米松抑制肾上腺静脉采血(AVS),肾上腺静脉与外周静脉皮质醇比值>6.5,以及醛固酮校正皮质醇侧化率>2.3,自主皮质醇分泌定位于右侧肾上腺腺瘤。她接受了右后腹膜后腹腔镜肾上腺切除术,高皮质醇血症在临床和生化方面得到缓解。术后,她需要氢化可的松治疗,4年后成功停用。结合醛固酮校正皮质醇侧化率使用地塞米松抑制AVS,可能会提高AVS的诊断准确性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eb9/12457819/fbc45c5c3537/luaf192f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eb9/12457819/6f98ae75f35c/luaf192f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eb9/12457819/fbc45c5c3537/luaf192f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eb9/12457819/6f98ae75f35c/luaf192f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eb9/12457819/fbc45c5c3537/luaf192f2.jpg

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本文引用的文献

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J Clin Endocrinol Metab. 2025 Aug 7;110(9):e2985-e2993. doi: 10.1210/clinem/dgae896.
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Ga-pentixafor PET/CT in the localization diagnosis of primary aldosteronism concurrent subclinical cushing's syndrsome: two case reports.Ga-苯并戊烯托 PET/CT 在原发性醛固酮增多症并发亚临床库欣综合征定位诊断中的应用:两例报告。
Endocrine. 2024 Sep;85(3):1398-1406. doi: 10.1007/s12020-024-03865-6. Epub 2024 Jun 24.
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The value of plasma metanephrine measurements during adrenal vein sampling.
肾上腺静脉采血期间血浆间甲肾上腺素测量的价值
Endocr Connect. 2024 Jan 12;13(2). doi: 10.1530/EC-23-0300. Print 2024 Feb 1.
4
Accuracy of Gallium-68 Pentixafor Positron Emission Tomography-Computed Tomography for Subtyping Diagnosis of Primary Aldosteronism.镓-68 吡咯烷酮[¹¹C]戊烷多靶素正电子发射断层扫描-计算机断层扫描用于原发性醛固酮增多症的亚型诊断的准确性。
JAMA Netw Open. 2023 Feb 1;6(2):e2255609. doi: 10.1001/jamanetworkopen.2022.55609.
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Adrenal venous sampling for lateralization of cortisol hypersecretion in patients with bilateral adrenal masses.双侧肾上腺肿块患者肾上腺静脉采血用于皮质醇分泌过多的定位诊断
Clin Endocrinol (Oxf). 2023 Feb;98(2):177-189. doi: 10.1111/cen.14833. Epub 2022 Nov 1.
6
Usefulness of 68 Ga-Pentixafor PET/CT on Diagnosis and Management of Cushing Syndrome.68Ga- 培哚普利 PET/CT 在库欣综合征诊断和治疗中的应用
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Utility of Epinephrine Levels in Determining Adrenal Vein Cannulation During Adrenal Venous Sampling for Primary Aldosteronism.在原发性醛固酮增多症的肾上腺静脉取样中,肾上腺素水平在确定肾上腺静脉插管中的作用。
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Functional Characterization of Adrenocortical Masses in Nononcologic Patients Using Ga-Pentixafor.使用镓-喷替沙氟对非肿瘤患者肾上腺皮质肿块进行功能表征
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Extensive clinical experience: Hypothalamic-pituitary-adrenal axis recovery after adrenalectomy for corticotropin-independent cortisol excess.广泛的临床经验:促肾上腺皮质激素非依赖性皮质醇增多症患者肾上腺切除术后下丘脑-垂体-肾上腺轴的恢复。
Clin Endocrinol (Oxf). 2018 Dec;89(6):721-733. doi: 10.1111/cen.13803. Epub 2018 Jul 23.