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原发性醛固酮增多症:单侧肾上腺切除术后的功能性组织病理学及长期随访

Primary aldosteronism: functional histopathology and long-term follow-up after unilateral adrenalectomy.

作者信息

Volpe Cristina, Hamberger Bertil, Höög Anders, Mukai Kuniaki, Calissendorff Jan, Wahrenberg Hans, Zedenius Jan, Thorén Marja

机构信息

Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden; Department of Internal Medicine, Division of Endocrinology, Södersjukhuset, Stockholm, Sweden.

出版信息

Clin Endocrinol (Oxf). 2015 May;82(5):639-47. doi: 10.1111/cen.12645. Epub 2014 Dec 19.

Abstract

OBJECTIVES

To investigate the long-term outcome after unilateral adrenalectomy in patients with primary aldosteronism (PA) and to establish the role of functional pathology for the final diagnosis of aldosterone-producing adenoma (APA) or hyperplasia.

DESIGN

A single-centre, retrospective cohort study in a hospital setting.

PATIENTS

Consecutive patients with PA, n = 120, who underwent unilateral adrenalectomy between 1985 and 2010. Preoperative and postoperative data were analysed. To indicate the site of aldosterone secretion in the resected adrenal, we added functional methods to routine histopathology, using in situ hybridization and immunohistochemistry to detect the presence of enzymes needed for aldosterone (CYP11B2) and cortisol (CYP11B1, CYP17A1) synthesis.

RESULTS

The median follow-up was 5 years and the cure rate of PA 91%. Hypertension was improved in 97% and normalized in 38%. Functional histopathology changed the final diagnosis from APA to hyperplasia in 6 cases (7%). Five of these had no expression of or staining for aldosterone synthase in the adenoma, but only in nodules in the adjacent cortex. All except one APA patient were cured of PA. They had lower preoperative serum potassium and higher 24-h urinary aldosterone than patients with hyperplasia. Among patients with hyperplasia 16 of 26 were cured.

CONCLUSIONS

Most patients were cured of PA by unilateral adrenalectomy. Almost all noncured benefitted from the operation as the blood pressure improved. Functional histopathology proved helpful in the distinction between APA and hyperplasia, and we recommend that functional histopathology should be added to routine histopathology to improve the diagnostic evaluation and aid in tailoring the follow-up.

摘要

目的

研究原发性醛固酮增多症(PA)患者单侧肾上腺切除术后的长期预后,并确定功能病理学在醛固酮瘤(APA)或增生的最终诊断中的作用。

设计

在医院环境中进行的单中心回顾性队列研究。

患者

1985年至2010年间接受单侧肾上腺切除术的连续PA患者,共120例。分析术前和术后数据。为了表明切除肾上腺中醛固酮分泌的部位,我们在常规组织病理学中增加了功能方法,使用原位杂交和免疫组织化学检测醛固酮(CYP11B2)和皮质醇(CYP11B1、CYP17A1)合成所需酶的存在。

结果

中位随访时间为5年,PA的治愈率为91%。97%的患者高血压得到改善,38%的患者血压恢复正常。功能组织病理学使6例(7%)患者的最终诊断从APA变为增生。其中5例腺瘤中醛固酮合酶无表达或染色,但仅在相邻皮质的结节中有表达。除1例APA患者外,所有患者的PA均得到治愈。他们术前血清钾较低,24小时尿醛固酮高于增生患者。在增生患者中,26例中有16例得到治愈。

结论

大多数PA患者通过单侧肾上腺切除术治愈。几乎所有未治愈的患者血压均有改善,手术有益。功能组织病理学有助于区分APA和增生,我们建议在常规组织病理学中增加功能组织病理学,以改善诊断评估并辅助制定随访方案。

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