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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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.
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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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