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原发性胸腺类癌伴库欣综合征

Primary thymic carcinoid with Cushing's syndrome.

作者信息

Wollensak G, Herbst E W, Beck A, Schaefer H E

机构信息

Department of Pathology, University of Freiburg, Federal Republic of Germany.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1992;420(2):191-5. doi: 10.1007/BF02358812.

DOI:10.1007/BF02358812
PMID:1372458
Abstract

In a 52-year-old Caucasian man osteopoikilosis had been misdiagnosed roentgenologically 2 years before his death. Gradually he developed Cushing's syndrome and ultimately superior vena caval obstruction. At autopsy a primary thymic carcinoid with extensive osteoblastic bone metastasis was found. Immunohistochemically the tumor was shown to be positive for adrenocorticotropic hormone (ACTH), cytokeratin (KL1), neuron-specific enolase, synaptophysin, chromogranin and glucagon. Remarkably the tumour was negative for serotonin despite high urinary hydroxyindolacetic acid levels. Bilateral hyperplasia of the adrenal cortex was found. The adenohypophysis showed a considerable reduction of ACTH-producing cells and numerous Crooke's cells with a characteristic immunohistochemical pattern.

摘要

一名52岁的白种男性,骨斑点症在其去世前2年被放射学误诊。他逐渐发展为库欣综合征,最终出现上腔静脉阻塞。尸检发现原发性胸腺类癌伴广泛成骨性骨转移。免疫组化显示肿瘤促肾上腺皮质激素(ACTH)、细胞角蛋白(KL1)、神经元特异性烯醇化酶、突触素、嗜铬粒蛋白和胰高血糖素呈阳性。值得注意的是,尽管尿中羟吲哚乙酸水平很高,但肿瘤5-羟色胺呈阴性。发现双侧肾上腺皮质增生。腺垂体促肾上腺皮质激素分泌细胞明显减少,有许多具有特征性免疫组化模式的克鲁克细胞。

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1
Primary thymic carcinoid with Cushing's syndrome.原发性胸腺类癌伴库欣综合征
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[A case of ACTH producing thymic carcinoid with Cushing's syndrome].[一例伴库欣综合征的促肾上腺皮质激素分泌性胸腺类癌病例]
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[Ectopic ACTH- or CRH-secreting tumors in Cushing's syndrome].[库欣综合征中分泌异位促肾上腺皮质激素(ACTH)或促肾上腺皮质激素释放激素(CRH)的肿瘤]
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引用本文的文献

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Ectopic Cushing's syndrome due to thymic neuroendocrine tumours: a systematic review.胸腺神经内分泌肿瘤导致异位库欣综合征:系统综述。
Rev Endocr Metab Disord. 2021 Dec;22(4):1041-1056. doi: 10.1007/s11154-021-09660-2. Epub 2021 May 7.
2
CT/MRI of neuroendocrine tumours.神经内分泌肿瘤的CT/MRI检查
Cancer Imaging. 2006 Oct 31;6(Spec No A):S163-77. doi: 10.1102/1470-7330.2006.9037.
3
Carcinoid tumours of the thymus.胸腺类癌肿瘤

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Carcinoid tumour of the thymus with Cushing's syndrome.胸腺类癌伴库欣综合征
Pathology. 1980 Jul;12(3):487-94. doi: 10.3109/00313028009077114.
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Thymic carcinoid tumor with Cushing's syndrome. Report of a case with electron microscopic and immunoperoxidase studies for neuron-specific enolase and corticotropin.胸腺类癌伴库欣综合征。一例经电子显微镜及神经元特异性烯醇化酶和促肾上腺皮质激素免疫过氧化物酶研究的病例报告。
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