@ARTICLE{10.3389/fendo.2019.00936, AUTHOR={Liu, Siyue and Liu, Zhelong and Chen, Fuqiong and Xu, Weijie and 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/articles/10.3389/fendo.2019.00936}, DOI={10.3389/fendo.2019.00936}, ISSN={1664-2392}, ABSTRACT={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.} }