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...

Descripción completa

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
_version_ 1783491796087603200
author Liu, Siyue
Liu, Zhelong
Chen, Fuqiong
Xu, Weijie
Yuan, Gang
author_facet Liu, Siyue
Liu, Zhelong
Chen, Fuqiong
Xu, Weijie
Yuan, Gang
author_sort Liu, Siyue
collection PubMed
description 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.
format Online
Article
Text
id pubmed-6985366
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-69853662020-02-07 Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature Liu, Siyue Liu, Zhelong Chen, Fuqiong Xu, Weijie Yuan, Gang Front Endocrinol (Lausanne) Endocrinology 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. Frontiers Media S.A. 2020-01-21 /pmc/articles/PMC6985366/ /pubmed/32038491 http://dx.doi.org/10.3389/fendo.2019.00936 Text en Copyright © 2020 Liu, Liu, Chen, Xu and Yuan. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Liu, Siyue
Liu, Zhelong
Chen, Fuqiong
Xu, Weijie
Yuan, Gang
Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title_full Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title_fullStr Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title_full_unstemmed Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title_short Adrenocorticotropic Hormone-Producing Paraganglioma With Low Plasma ACTH Level: A Case Report and Review of the Literature
title_sort adrenocorticotropic hormone-producing paraganglioma with low plasma acth level: a case report and review of the literature
topic Endocrinology
url 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
work_keys_str_mv AT liusiyue adrenocorticotropichormoneproducingparagangliomawithlowplasmaacthlevelacasereportandreviewoftheliterature
AT liuzhelong adrenocorticotropichormoneproducingparagangliomawithlowplasmaacthlevelacasereportandreviewoftheliterature
AT chenfuqiong adrenocorticotropichormoneproducingparagangliomawithlowplasmaacthlevelacasereportandreviewoftheliterature
AT xuweijie adrenocorticotropichormoneproducingparagangliomawithlowplasmaacthlevelacasereportandreviewoftheliterature
AT yuangang adrenocorticotropichormoneproducingparagangliomawithlowplasmaacthlevelacasereportandreviewoftheliterature

Ejemplares similares