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胸腺类癌伴促肾上腺皮质激素分泌性异位库欣综合征及空蝶鞍

THYMIC CARCINOID WITH ADRENOCORTICOTROPIC HORMONE-PRODUCING ECTOPIC CUSHING SYNDROME AND EMPTY SELLA.

作者信息

Labadzhyan Artak, Kim Se-Min, Rhyu Jane, Araki Takako, Mahtabifard Ali, Melmed Shlomo

机构信息

Pituitary Center, Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, California, United States of America.

Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, University of Minnesota, Minneapolis, Minnesota, United States of America.

出版信息

AACE Clin Case Rep. 2018 Sep-Oct;4(5):e375-e378. doi: 10.4158/ACCR-2018-0045. Epub 2018 May 1.

Abstract

OBJECTIVE

Ectopic Cushing's syndrome secondary to thymic carcinoid is a rare disorder that may be difficult to diagnose and manage.

METHODS

We describe a case of severe Cushing's syndrome secondary to a large adrenocorticotropic hormone (ACTH) producing thymic carcinoid in a patient with history of primary hyperaldosteronism.

RESULTS

A 43-year-old female with a 20-year history of an aldosterone-secreting adrenocortical adenoma status post right adrenalectomy presented with acute onset of proximal muscle weakness, swelling, facial hirsutism, and severe hypokalemia. Ectopic Cushing's Syndrome was suspected based on the sudden symptom onset and markedly elevated 24-hr urine cortisol and ACTH levels. MRI revealed an empty pituitary sella and a large (7.3 cm) mediastinal mass visible on chest CT. The mass was resected by video-assisted thoracoscopic surgery, resulting in resolution of symptoms and cortisol levels. Pathology assessment confirmed well-differentiated thymic carcinoid with positive ACTH staining.

CONCLUSION

The case highlights clinical features, challenges in diagnostic work up, treatment modalities, and associated endocrine findings in a thymic carcinoid abutting the heart and presenting with ectopic ACTH secretion.

摘要

目的

继发于胸腺类癌的异位库欣综合征是一种罕见疾病,可能难以诊断和处理。

方法

我们描述了一例严重库欣综合征病例,该患者既往有原发性醛固酮增多症病史,继发于产生大量促肾上腺皮质激素(ACTH)的胸腺类癌。

结果

一名43岁女性,有分泌醛固酮的肾上腺皮质腺瘤病史20年,右侧肾上腺切除术后,出现急性近端肌无力、肿胀、面部多毛和严重低钾血症。基于症状突然发作以及24小时尿皮质醇和ACTH水平显著升高,怀疑为异位库欣综合征。MRI显示垂体空蝶鞍,胸部CT可见一个大的(7.3 cm)纵隔肿块。通过电视辅助胸腔镜手术切除肿块,症状和皮质醇水平得以缓解。病理评估证实为高分化胸腺类癌,ACTH染色阳性。

结论

该病例突出了胸腺类癌紧邻心脏并伴有异位ACTH分泌时的临床特征、诊断检查中的挑战、治疗方式以及相关内分泌表现。

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