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Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma

CONTEXT: Information about adrenal medullary hyperplasia (AMH) is scarce. OBJECTIVE: To study a large cohort of AMHs. DESIGN, SETTING, AND PARTICIPANTS: Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately....

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Autores principales: Falhammar, Henrik, Stenman, Adam, Calissendorff, Jan, Juhlin, Carl Christofer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6676072/
https://www.ncbi.nlm.nih.gov/pubmed/31384714
http://dx.doi.org/10.1210/js.2019-00200
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author Falhammar, Henrik
Stenman, Adam
Calissendorff, Jan
Juhlin, Carl Christofer
author_facet Falhammar, Henrik
Stenman, Adam
Calissendorff, Jan
Juhlin, Carl Christofer
author_sort Falhammar, Henrik
collection PubMed
description CONTEXT: Information about adrenal medullary hyperplasia (AMH) is scarce. OBJECTIVE: To study a large cohort of AMHs. DESIGN, SETTING, AND PARTICIPANTS: Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately. RESULTS: Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences. CONCLUSION: A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.
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spelling pubmed-66760722019-08-05 Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma Falhammar, Henrik Stenman, Adam Calissendorff, Jan Juhlin, Carl Christofer J Endocr Soc Clinical Research Articles CONTEXT: Information about adrenal medullary hyperplasia (AMH) is scarce. OBJECTIVE: To study a large cohort of AMHs. DESIGN, SETTING, AND PARTICIPANTS: Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately. RESULTS: Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences. CONCLUSION: A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up. Endocrine Society 2019-06-11 /pmc/articles/PMC6676072/ /pubmed/31384714 http://dx.doi.org/10.1210/js.2019-00200 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Clinical Research Articles
Falhammar, Henrik
Stenman, Adam
Calissendorff, Jan
Juhlin, Carl Christofer
Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title_full Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title_fullStr Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title_full_unstemmed Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title_short Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma
title_sort presentation, treatment, histology, and outcomes in adrenal medullary hyperplasia compared with pheochromocytoma
topic Clinical Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6676072/
https://www.ncbi.nlm.nih.gov/pubmed/31384714
http://dx.doi.org/10.1210/js.2019-00200
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