Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature

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 immunofluo...

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Detalles Bibliográficos
Autores principales: Liu, Siyue, Liu, Zhelong, Chen, Fuqiong, Xu, Weijie, Yuan, Gang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Endocrinology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6985366/
https://www.ncbi.nlm.nih.gov/pubmed/32038491
http://dx.doi.org/10.3389/fendo.2019.00936
Descripción
Sumario: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.

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