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Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center

Testicular adrenal rest tumors (TARTs) are a common complication in male patients with congenital adrenal hyperplasia (CAH). The aim of our cross-sectional cohort study is to estimate the frequency of TARTs with the correlation of genotype and disease control on tumor development. Thirty-five male p...

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Autores principales: Ortolano, Rita, Cassio, Alessandra, Alqaisi, Randa S., Candela, Egidio, Di Natale, Valeria, Assirelli, Valentina, Bernardini, Luca, Bortolamedi, Elisa, Cantarelli, Erika, Corcioni, Beniamino, Renzulli, Matteo, Balsamo, Antonio, Baronio, Federico
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10528159/
https://www.ncbi.nlm.nih.gov/pubmed/37761418
http://dx.doi.org/10.3390/children10091457
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author Ortolano, Rita
Cassio, Alessandra
Alqaisi, Randa S.
Candela, Egidio
Di Natale, Valeria
Assirelli, Valentina
Bernardini, Luca
Bortolamedi, Elisa
Cantarelli, Erika
Corcioni, Beniamino
Renzulli, Matteo
Balsamo, Antonio
Baronio, Federico
author_facet Ortolano, Rita
Cassio, Alessandra
Alqaisi, Randa S.
Candela, Egidio
Di Natale, Valeria
Assirelli, Valentina
Bernardini, Luca
Bortolamedi, Elisa
Cantarelli, Erika
Corcioni, Beniamino
Renzulli, Matteo
Balsamo, Antonio
Baronio, Federico
author_sort Ortolano, Rita
collection PubMed
description Testicular adrenal rest tumors (TARTs) are a common complication in male patients with congenital adrenal hyperplasia (CAH). The aim of our cross-sectional cohort study is to estimate the frequency of TARTs with the correlation of genotype and disease control on tumor development. Thirty-five male patients, aged 14–26 years, were included in the study, all followed by the same center of pediatric endocrinology in Bologna. We studied genotypes, hormonal profiles at different time intervals and testicular ultrasound. A logistic regression model with multivariant analysis was developed for the statistical analysis. TARTs were detected in 31.4% of the cases, 90.9% of them had a classic form with salt wasting, while 9.1% had a non-classic form. Additionally, a significant correlation between the incidence of TARTs and severity of genotype was detected. Patients with TARTs had markedly worse metabolic control on average (p = 0.027), reflected by high ACTH, 17OH progesterone, and overall delta4-androstenedione. In conclusion, a screening tool is mandatory, especially (but not exclusively) in patients with the most severe forms of CAH and poor endocrine control of the disease.
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spelling pubmed-105281592023-09-28 Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center Ortolano, Rita Cassio, Alessandra Alqaisi, Randa S. Candela, Egidio Di Natale, Valeria Assirelli, Valentina Bernardini, Luca Bortolamedi, Elisa Cantarelli, Erika Corcioni, Beniamino Renzulli, Matteo Balsamo, Antonio Baronio, Federico Children (Basel) Article Testicular adrenal rest tumors (TARTs) are a common complication in male patients with congenital adrenal hyperplasia (CAH). The aim of our cross-sectional cohort study is to estimate the frequency of TARTs with the correlation of genotype and disease control on tumor development. Thirty-five male patients, aged 14–26 years, were included in the study, all followed by the same center of pediatric endocrinology in Bologna. We studied genotypes, hormonal profiles at different time intervals and testicular ultrasound. A logistic regression model with multivariant analysis was developed for the statistical analysis. TARTs were detected in 31.4% of the cases, 90.9% of them had a classic form with salt wasting, while 9.1% had a non-classic form. Additionally, a significant correlation between the incidence of TARTs and severity of genotype was detected. Patients with TARTs had markedly worse metabolic control on average (p = 0.027), reflected by high ACTH, 17OH progesterone, and overall delta4-androstenedione. In conclusion, a screening tool is mandatory, especially (but not exclusively) in patients with the most severe forms of CAH and poor endocrine control of the disease. MDPI 2023-08-26 /pmc/articles/PMC10528159/ /pubmed/37761418 http://dx.doi.org/10.3390/children10091457 Text en © 2023 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 Article
Ortolano, Rita
Cassio, Alessandra
Alqaisi, Randa S.
Candela, Egidio
Di Natale, Valeria
Assirelli, Valentina
Bernardini, Luca
Bortolamedi, Elisa
Cantarelli, Erika
Corcioni, Beniamino
Renzulli, Matteo
Balsamo, Antonio
Baronio, Federico
Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title_full Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title_fullStr Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title_full_unstemmed Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title_short Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center
title_sort testicular adrenal rest tumors in congenital adrenal hyperplasia: study of a cohort of patients from a single italian center
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10528159/
https://www.ncbi.nlm.nih.gov/pubmed/37761418
http://dx.doi.org/10.3390/children10091457
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