Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour

Ectopic production of adrenocorticotropic hormone (ACTH) by gastrointestinal neuroendocrine tumours (NETs) is relatively uncommon. We report a rare case of a liver metastatic G1 low-grade NET of the intestine that induced hypercortisolism after surgical resection. A 50-year-old man was admitted for...

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Main Authors: Laurent Mineur, Rania Boustany, Léa Vazquez
Format: Article
Language:English
Published: Karger Publishers 2021-09-01
Series:Case Reports in Oncology
Subjects:
Online Access:https://www.karger.com/Article/FullText/518316
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author Laurent Mineur
Rania Boustany
Léa Vazquez
author_facet Laurent Mineur
Rania Boustany
Léa Vazquez
author_sort Laurent Mineur
collection DOAJ
description Ectopic production of adrenocorticotropic hormone (ACTH) by gastrointestinal neuroendocrine tumours (NETs) is relatively uncommon. We report a rare case of a liver metastatic G1 low-grade NET of the intestine that induced hypercortisolism after surgical resection. A 50-year-old man was admitted for an intestinal obstruction caused by a tumour of the intestine. Paraneoplastic Cushing syndrome was diagnosed more than a year later following the appearance of cushingoid symptoms, despite stable disease according to RECIST criteria but chromogranin A increase. Ketoconazole and sandostatin medical treatment and liver chemoembolization never managed to control the hypercortisolism unlike the bilateral adrenalectomy. The identification and effective management of this uncommon statement of ectopic ACTH secretion is important to improve the patient’s prognosis and quality of life.
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spelling doaj.art-7b89a3e004974d13ae0c5563c788087b2022-12-21T21:24:26ZengKarger PublishersCase Reports in Oncology1662-65752021-09-011431407141310.1159/000518316518316Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine TumourLaurent MineurRania BoustanyLéa VazquezEctopic production of adrenocorticotropic hormone (ACTH) by gastrointestinal neuroendocrine tumours (NETs) is relatively uncommon. We report a rare case of a liver metastatic G1 low-grade NET of the intestine that induced hypercortisolism after surgical resection. A 50-year-old man was admitted for an intestinal obstruction caused by a tumour of the intestine. Paraneoplastic Cushing syndrome was diagnosed more than a year later following the appearance of cushingoid symptoms, despite stable disease according to RECIST criteria but chromogranin A increase. Ketoconazole and sandostatin medical treatment and liver chemoembolization never managed to control the hypercortisolism unlike the bilateral adrenalectomy. The identification and effective management of this uncommon statement of ectopic ACTH secretion is important to improve the patient’s prognosis and quality of life.https://www.karger.com/Article/FullText/518316ectopic cushing syndromeneuroendocrine tumourswho grade 1paraneoplastic syndromeschromogranin a
spellingShingle Laurent Mineur
Rania Boustany
Léa Vazquez
Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
Case Reports in Oncology
ectopic cushing syndrome
neuroendocrine tumours
who grade 1
paraneoplastic syndromes
chromogranin a
title Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
title_full Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
title_fullStr Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
title_full_unstemmed Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
title_short Paraneoplastic Cushing Syndrome in Gastrointestinal Neuroendocrine Tumour
title_sort paraneoplastic cushing syndrome in gastrointestinal neuroendocrine tumour
topic ectopic cushing syndrome
neuroendocrine tumours
who grade 1
paraneoplastic syndromes
chromogranin a
url https://www.karger.com/Article/FullText/518316
work_keys_str_mv AT laurentmineur paraneoplasticcushingsyndromeingastrointestinalneuroendocrinetumour
AT raniaboustany paraneoplasticcushingsyndromeingastrointestinalneuroendocrinetumour
AT leavazquez paraneoplasticcushingsyndromeingastrointestinalneuroendocrinetumour