Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre.
<h4>Purpose</h4>Ectopic Cushing Syndrome (EAS) is a rare condition responsible for about 5-20% of all Cushing syndrome cases. It increases the mortality of affected patients thus finding and removal of the ACTH-producing source allows for curing or reduction of symptoms and serum cortiso...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2020-01-01
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| Series: | PLoS ONE |
| Online Access: | https://doi.org/10.1371/journal.pone.0242679 |
| _version_ | 1819292599827038208 |
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| author | Joanna Ewelina Paleń-Tytko Elwira Maria Przybylik-Mazurek Ewelina Joanna Rzepka Dorota Magdalena Pach Anna Stanisława Sowa-Staszczak Aleksandra Gilis-Januszewska Alicja Bronisława Hubalewska-Dydejczyk |
| author_facet | Joanna Ewelina Paleń-Tytko Elwira Maria Przybylik-Mazurek Ewelina Joanna Rzepka Dorota Magdalena Pach Anna Stanisława Sowa-Staszczak Aleksandra Gilis-Januszewska Alicja Bronisława Hubalewska-Dydejczyk |
| author_sort | Joanna Ewelina Paleń-Tytko |
| collection | DOAJ |
| description | <h4>Purpose</h4>Ectopic Cushing Syndrome (EAS) is a rare condition responsible for about 5-20% of all Cushing syndrome cases. It increases the mortality of affected patients thus finding and removal of the ACTH-producing source allows for curing or reduction of symptoms and serum cortisol levels. The aim of this study is to present a 20-year experience in the diagnosis and clinical course of patients with EAS in a single Clinical Centre in Southern Poland as well as a comparison of clinical course and outcomes depending on the source of ectopic ACTH production-especially neuroendocrine tumors with other neoplasms.<h4>Methods</h4>Twenty-four patients were involved in the clinical study with EAS diagnosed at the Department of Endocrinology between years 2000 and 2018. The diagnosis of EAS was based on the clinical presentation, hypercortisolemia with high ACTH levels, high dose dexamethasone suppression test and/or corticotropin-releasing hormone tests. To find the source of ACTH various imaging studies were performed.<h4>Results</h4>Half of the patients were diagnosed with neuroendocrine tumors, whereby muscle weakness was the leading symptom. Typical cushingoid appearance was seen in merely a few patients, and weight loss was more common than weight gain. Patients with neuroendocrine tumors had significantly higher midnight cortisol levels than the rest of the group. Among patients with infections, we observed a significantly higher concentrations of cortisol 2400 levels in gastroenteropancreatic neuroendocrine tumors. Chromogranin A correlated significantly with potassium in patients with neuroendocrine tumors and there was a significant correlation between ACTH level and severity of hypokalemia.<h4>Conclusion</h4>EAS is not common, but if it occurs it increases the mortality of patients; therefore, it should be taken into consideration in the case of coexistence of severe hypokalemia with hypertension and muscle weakness, especially when weight loss occurs. Because the diagnosis of gastroenteropancreatic neuroendocrine tumor worsens the prognosis-special attention should be paid to these patients. |
| first_indexed | 2024-12-24T03:57:06Z |
| format | Article |
| id | doaj.art-a0d21032e546426fab8e4f1b36c5e0d9 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-24T03:57:06Z |
| publishDate | 2020-01-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-a0d21032e546426fab8e4f1b36c5e0d92022-12-21T17:16:25ZengPublic Library of Science (PLoS)PLoS ONE1932-62032020-01-011511e024267910.1371/journal.pone.0242679Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre.Joanna Ewelina Paleń-TytkoElwira Maria Przybylik-MazurekEwelina Joanna RzepkaDorota Magdalena PachAnna Stanisława Sowa-StaszczakAleksandra Gilis-JanuszewskaAlicja Bronisława Hubalewska-Dydejczyk<h4>Purpose</h4>Ectopic Cushing Syndrome (EAS) is a rare condition responsible for about 5-20% of all Cushing syndrome cases. It increases the mortality of affected patients thus finding and removal of the ACTH-producing source allows for curing or reduction of symptoms and serum cortisol levels. The aim of this study is to present a 20-year experience in the diagnosis and clinical course of patients with EAS in a single Clinical Centre in Southern Poland as well as a comparison of clinical course and outcomes depending on the source of ectopic ACTH production-especially neuroendocrine tumors with other neoplasms.<h4>Methods</h4>Twenty-four patients were involved in the clinical study with EAS diagnosed at the Department of Endocrinology between years 2000 and 2018. The diagnosis of EAS was based on the clinical presentation, hypercortisolemia with high ACTH levels, high dose dexamethasone suppression test and/or corticotropin-releasing hormone tests. To find the source of ACTH various imaging studies were performed.<h4>Results</h4>Half of the patients were diagnosed with neuroendocrine tumors, whereby muscle weakness was the leading symptom. Typical cushingoid appearance was seen in merely a few patients, and weight loss was more common than weight gain. Patients with neuroendocrine tumors had significantly higher midnight cortisol levels than the rest of the group. Among patients with infections, we observed a significantly higher concentrations of cortisol 2400 levels in gastroenteropancreatic neuroendocrine tumors. Chromogranin A correlated significantly with potassium in patients with neuroendocrine tumors and there was a significant correlation between ACTH level and severity of hypokalemia.<h4>Conclusion</h4>EAS is not common, but if it occurs it increases the mortality of patients; therefore, it should be taken into consideration in the case of coexistence of severe hypokalemia with hypertension and muscle weakness, especially when weight loss occurs. Because the diagnosis of gastroenteropancreatic neuroendocrine tumor worsens the prognosis-special attention should be paid to these patients.https://doi.org/10.1371/journal.pone.0242679 |
| spellingShingle | Joanna Ewelina Paleń-Tytko Elwira Maria Przybylik-Mazurek Ewelina Joanna Rzepka Dorota Magdalena Pach Anna Stanisława Sowa-Staszczak Aleksandra Gilis-Januszewska Alicja Bronisława Hubalewska-Dydejczyk Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. PLoS ONE |
| title | Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. |
| title_full | Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. |
| title_fullStr | Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. |
| title_full_unstemmed | Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. |
| title_short | Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre. |
| title_sort | ectopic acth syndrome of different origin diagnostic approach and clinical outcome experience of one clinical centre |
| url | https://doi.org/10.1371/journal.pone.0242679 |
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