血浆促肾上腺皮质激素水平低的促肾上腺皮质激素分泌型副神经节瘤：一例报告并文献复习
Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature.
作者信息
Liu Siyue, Liu Zhelong, Chen Fuqiong, Xu Weijie, Yuan Gang
机构信息
Department of Endocrinology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China.
出版信息
Front Endocrinol (Lausanne). 2020 Jan 21;10:936. doi: 10.3389/fendo.2019.00936. eCollection 2019.
Ectopic adrenocorticotropic hormone (ACTH) syndrome caused by paraganglioma is extremely rare. It usually accompanied by high or normal plasma ACTH level. Here we described a male who presented with ectopic ACTH-producing paraganglioma and a low plasma ACTH level. Immunohistochemistry and immunofluorescence confirmed ACTH production in focal paraganglioma cells. This unusual case expanded the spectrum of ACTH-dependent Cushing's syndrome and revealed a potential mechanism of this unique clinical phenotype. Besides, the literature concerning ACTH-producing paraganglioma is reviewed.
由副神经节瘤引起的异位促肾上腺皮质激素（ACTH）综合征极为罕见。它通常伴有血浆ACTH水平升高或正常。在此，我们描述了一名患有分泌异位ACTH的副神经节瘤且血浆ACTH水平较低的男性患者。免疫组织化学和免疫荧光证实了局灶性副神经节瘤细胞中ACTH的产生。这一罕见病例扩展了ACTH依赖性库欣综合征的范围，并揭示了这种独特临床表型的潜在机制。此外，还对有关分泌ACTH的副神经节瘤的文献进行了综述。
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