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Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling
Background: Malignant mixed corticomedullary adrenal tumors (MCMTs) are extremely rare, with limited cases reported in the literature. The pathophysiology of malignant MCMTs is not well understood; the most prevailing theories are that it is a composite tumor of embryologically derived mesodermal (a...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9680374/ https://www.ncbi.nlm.nih.gov/pubmed/36412675 http://dx.doi.org/10.3390/clinpract12060096 |
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author | Ullah, Asad Mohamed, Farah Ayman Elsaid Khan, Jaffar Tracy, Katharine Khan, Muhabat Mohsen, Samiha Yasinzai, Abdul Qahar Khan Badini, Kaleemullah Sobash, Philip T. Heneidi, Saleh Karim, Nagla Abdel |
author_facet | Ullah, Asad Mohamed, Farah Ayman Elsaid Khan, Jaffar Tracy, Katharine Khan, Muhabat Mohsen, Samiha Yasinzai, Abdul Qahar Khan Badini, Kaleemullah Sobash, Philip T. Heneidi, Saleh Karim, Nagla Abdel |
author_sort | Ullah, Asad |
collection | PubMed |
description | Background: Malignant mixed corticomedullary adrenal tumors (MCMTs) are extremely rare, with limited cases reported in the literature. The pathophysiology of malignant MCMTs is not well understood; the most prevailing theories are that it is a composite tumor of embryologically derived mesodermal (adrenal cortex) and neural crest (medulla) origin, perpetuating as two distinct cell lines forming a singular mass. Clinical features and laboratory diagnosis are associated with hypersecretions of the adrenal cortex and medulla. Surgical resection is curative in an isolated tumor. We reviewed and compared cases in the literature highlighting the pathogenesis and genetics of benign and malignant MCMT. Methods: Comprehensive literature analysis was conducted on PubMed and all the cases of mixed corticomedullary adrenal tumor published in English were included. Results: Most patients were female (73.1%) with a median age of 49 in women and 50 in men. Surgery was performed in all patients, and in four patients with malignant disease, chemotherapy was used as well. Clinically, most patients presented with hypertension (69%) followed by Cushing syndrome (42%) and diabetes (19%). Tumors often produced cortisol (74%), catecholamines (50%), and adrenocorticotrophic hormone (ACTH) (38%), with lower incidence of aldosterone- (7%) or dopamine (4%)-producing tumors. Immunohistochemical staining of 96% of cases showed Chromogranin-A (73%) and Synaptophysin (62%), followed by Inhibin-α (50%), Melan-A (31%), and S-100 (23%). Of the reported four cases with malignant disease, three showed a Ki-67 index of 40–50% with one showing less than 5%. Conclusion: Mixed corticomedullary adrenal tumors rarely present as a malignant disease requiring chemotherapy. Most MCMTs confer a good prognosis and respond well to surgical resection, though their pathogenesis is largely up to speculation because of limited data. Current theories regarding MCMT pathogenesis should be investigated further with genetic testing. Future research on MCMT may provide ways to guide physician diagnosis and subsequent treatment for refractory cases. |
format | Online Article Text |
id | pubmed-9680374 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96803742022-11-23 Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling Ullah, Asad Mohamed, Farah Ayman Elsaid Khan, Jaffar Tracy, Katharine Khan, Muhabat Mohsen, Samiha Yasinzai, Abdul Qahar Khan Badini, Kaleemullah Sobash, Philip T. Heneidi, Saleh Karim, Nagla Abdel Clin Pract Review Background: Malignant mixed corticomedullary adrenal tumors (MCMTs) are extremely rare, with limited cases reported in the literature. The pathophysiology of malignant MCMTs is not well understood; the most prevailing theories are that it is a composite tumor of embryologically derived mesodermal (adrenal cortex) and neural crest (medulla) origin, perpetuating as two distinct cell lines forming a singular mass. Clinical features and laboratory diagnosis are associated with hypersecretions of the adrenal cortex and medulla. Surgical resection is curative in an isolated tumor. We reviewed and compared cases in the literature highlighting the pathogenesis and genetics of benign and malignant MCMT. Methods: Comprehensive literature analysis was conducted on PubMed and all the cases of mixed corticomedullary adrenal tumor published in English were included. Results: Most patients were female (73.1%) with a median age of 49 in women and 50 in men. Surgery was performed in all patients, and in four patients with malignant disease, chemotherapy was used as well. Clinically, most patients presented with hypertension (69%) followed by Cushing syndrome (42%) and diabetes (19%). Tumors often produced cortisol (74%), catecholamines (50%), and adrenocorticotrophic hormone (ACTH) (38%), with lower incidence of aldosterone- (7%) or dopamine (4%)-producing tumors. Immunohistochemical staining of 96% of cases showed Chromogranin-A (73%) and Synaptophysin (62%), followed by Inhibin-α (50%), Melan-A (31%), and S-100 (23%). Of the reported four cases with malignant disease, three showed a Ki-67 index of 40–50% with one showing less than 5%. Conclusion: Mixed corticomedullary adrenal tumors rarely present as a malignant disease requiring chemotherapy. Most MCMTs confer a good prognosis and respond well to surgical resection, though their pathogenesis is largely up to speculation because of limited data. Current theories regarding MCMT pathogenesis should be investigated further with genetic testing. Future research on MCMT may provide ways to guide physician diagnosis and subsequent treatment for refractory cases. MDPI 2022-11-11 /pmc/articles/PMC9680374/ /pubmed/36412675 http://dx.doi.org/10.3390/clinpract12060096 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Ullah, Asad Mohamed, Farah Ayman Elsaid Khan, Jaffar Tracy, Katharine Khan, Muhabat Mohsen, Samiha Yasinzai, Abdul Qahar Khan Badini, Kaleemullah Sobash, Philip T. Heneidi, Saleh Karim, Nagla Abdel Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title | Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title_full | Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title_fullStr | Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title_full_unstemmed | Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title_short | Current Understanding of “Mixed Corticomedullary Adrenal Tumor” and an Insight into Genomic Profiling |
title_sort | current understanding of “mixed corticomedullary adrenal tumor” and an insight into genomic profiling |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9680374/ https://www.ncbi.nlm.nih.gov/pubmed/36412675 http://dx.doi.org/10.3390/clinpract12060096 |
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