title: Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency.
abstract: 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.
authors.affiliation: Endocrinology Department, Hedi Chaker Hospital, Sfax 3029, Tunisia.
authors.forename: Nadia
authors.initials: N
authors.lastname: Charfi
authors.forename: Mahdi
authors.initials: M
authors.lastname: Kamoun
authors.forename: Mouna
authors.initials: M
authors.lastname: Feki Mnif
authors.forename: Neila
authors.initials: N
authors.lastname: Mseddi
authors.forename: Fatma
authors.initials: F
authors.lastname: Mnif
authors.forename: Nozha
authors.initials: N
authors.lastname: Kallel
authors.forename: Basma
authors.initials: B
authors.lastname: Ben Naceur
authors.forename: Nabila
authors.initials: N
authors.lastname: Rekik
authors.forename: Hela
authors.initials: H
authors.lastname: Fourati
authors.forename: Emna
authors.initials: E
authors.lastname: Daoud
authors.forename: Zainab
authors.initials: Z
authors.lastname: Mnif
authors.forename: Mourad
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authors.lastname: Hadj Sliman
authors.forename: Tahia
authors.initials: T
authors.lastname: Sellami-Boudawara
authors.forename: Mohamed
authors.initials: M
authors.lastname: Abid