AUTHOR=Liu Siyue , Liu Zhelong , Chen Fuqiong , Xu Weijie , Yuan Gang 

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

JOURNAL=Frontiers in Endocrinology

VOLUME=10

YEAR=2020

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00936

DOI=10.3389/fendo.2019.00936

ISSN=1664-2392

ABSTRACT=<p>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.</p>