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Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma

Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magne...

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Autores principales: Serra, F, Duarte, S, Abreu, S, Marques, C, Cassis, J, Saraiva, M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3922001/
https://www.ncbi.nlm.nih.gov/pubmed/24616770
http://dx.doi.org/10.1530/EDM-13-0038
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author Serra, F
Duarte, S
Abreu, S
Marques, C
Cassis, J
Saraiva, M
author_facet Serra, F
Duarte, S
Abreu, S
Marques, C
Cassis, J
Saraiva, M
author_sort Serra, F
collection PubMed
description Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature. LEARNING POINTS: Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies. Most paraganglioma of the head and neck are not hormonally active. Nasal paraganglioma, especially ACTH producing, is a very rare tumour.
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spelling pubmed-39220012014-03-10 Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma Serra, F Duarte, S Abreu, S Marques, C Cassis, J Saraiva, M Endocrinol Diabetes Metab Case Rep Unique/Unexpected Symptoms or Presentations of a Disease Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature. LEARNING POINTS: Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies. Most paraganglioma of the head and neck are not hormonally active. Nasal paraganglioma, especially ACTH producing, is a very rare tumour. Bioscientifica Ltd 2013-09-16 2013 /pmc/articles/PMC3922001/ /pubmed/24616770 http://dx.doi.org/10.1530/EDM-13-0038 Text en © 2013 The authors This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License (http://creativecommons.org/licenses/by/3.0/deed.en_GB) .
spellingShingle Unique/Unexpected Symptoms or Presentations of a Disease
Serra, F
Duarte, S
Abreu, S
Marques, C
Cassis, J
Saraiva, M
Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title_full Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title_fullStr Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title_full_unstemmed Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title_short Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma
title_sort cushing's syndrome due to ectopic acth production by a nasal paraganglioma
topic Unique/Unexpected Symptoms or Presentations of a Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3922001/
https://www.ncbi.nlm.nih.gov/pubmed/24616770
http://dx.doi.org/10.1530/EDM-13-0038
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