Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may...

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Main Authors: Nadia Charfi, Mahdi Kamoun, Mouna Feki Mnif, Neila Mseddi, Fatma Mnif, Nozha Kallel, Basma Ben Naceur, Nabila Rekik, Hela Fourati, Emna Daoud, Zainab Mnif, Mourad Hadj Sliman, Tahia Sellami-Boudawara, Mohamed Abid
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Urology
Online Access:http://dx.doi.org/10.1155/2012/648643
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author Nadia Charfi
Mahdi Kamoun
Mouna Feki Mnif
Neila Mseddi
Fatma Mnif
Nozha Kallel
Basma Ben Naceur
Nabila Rekik
Hela Fourati
Emna Daoud
Zainab Mnif
Mourad Hadj Sliman
Tahia Sellami-Boudawara
Mohamed Abid
author_facet Nadia Charfi
Mahdi Kamoun
Mouna Feki Mnif
Neila Mseddi
Fatma Mnif
Nozha Kallel
Basma Ben Naceur
Nabila Rekik
Hela Fourati
Emna Daoud
Zainab Mnif
Mourad Hadj Sliman
Tahia Sellami-Boudawara
Mohamed Abid
author_sort Nadia Charfi
collection DOAJ
description Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (LCTs) are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.
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institution Kabale University
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publishDate 2012-01-01
publisher Wiley
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series Case Reports in Urology
spelling doaj-art-afa727884a2e441482589a323de24c562025-02-03T01:30:59ZengWileyCase Reports in Urology2090-696X2090-69782012-01-01201210.1155/2012/648643648643Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase DeficiencyNadia Charfi0Mahdi Kamoun1Mouna Feki Mnif2Neila Mseddi3Fatma Mnif4Nozha Kallel5Basma Ben Naceur6Nabila Rekik7Hela Fourati8Emna Daoud9Zainab Mnif10Mourad Hadj Sliman11Tahia Sellami-Boudawara12Mohamed Abid13Endocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaDepartment of Radiology, Hedi Chaker Hospital, Sfax 3029, TunisiaDepartment of Radiology, Hedi Chaker Hospital, Sfax 3029, TunisiaDepartment of Radiology, Hedi Chaker Hospital, Sfax 3029, TunisiaDepartment of Urology, Habib Bourguiba Hospital, Sfax 3029, TunisiaAnatomic Pathology Department, Habib Bourguiba Hospital, Sfax 3029, TunisiaEndocrinology Department, Hedi Chaker Hospital, Sfax 3029, TunisiaCongenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (LCTs) are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.http://dx.doi.org/10.1155/2012/648643
spellingShingle Nadia Charfi
Mahdi Kamoun
Mouna Feki Mnif
Neila Mseddi
Fatma Mnif
Nozha Kallel
Basma Ben Naceur
Nabila Rekik
Hela Fourati
Emna Daoud
Zainab Mnif
Mourad Hadj Sliman
Tahia Sellami-Boudawara
Mohamed Abid
Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
Case Reports in Urology
title Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
title_full Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
title_fullStr Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
title_full_unstemmed Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
title_short Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency
title_sort leydig cell tumor associated with testicular adrenal rest tumors in a patient with congenital adrenal hyperplasia due to 11β hydroxylase deficiency
url http://dx.doi.org/10.1155/2012/648643
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