Testicular Adrenal Rest Tumor Mimicking Leydig Cell Tumor in a Patient with Congenital Adrenal Hyperplasia
نویسندگان
چکیده
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder characterized by enzyme defects in the steroidogenic pathways. Testicular adrenal rest tumors (TARTs) are may have serious consequences in patients with CAH. They probably develop from ectopic remnants of intratesticular adrenal tissue, which might be stimulated by the rise of adrenocorticotropic hormone (ACTH). Their histological differentiation from Leydig-cell tumors is extremely difficult.[1]
منابع مشابه
A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
21-hydroxylase deficiency (21-OHD) caused congenital adrenal hyperplasia (CAH) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO) synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs) are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The p...
متن کاملMetachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)
A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and me...
متن کاملPrimary testicular abnormalities causing precocious puberty Leydig cell tumor, Leydig cell hyperplasia, and adrenal rest tumor.
The child with testicular enlargement in the absence of gonadotrophin stimulation presents a difficult diagnostic dilemma. Leydig cell tumors, Leydig cell hyperplasia, and tumors of adrenal rest tissue are the primary etiologic considerations. Because of considerable overlap in clinical presentation, careful biochemical and histologic evaluations are necessary to make the diagnosis. These shoul...
متن کاملTesticular Adrenal Rest Tumor in 11-Beta-Hydroxylase Deficiency Driven Congenital Adrenal Hyperplasia
Testicular adrenal rest tumors (TART) occur often as asymptomatic nodules in corticotropin-dependent lesions aberrant adrenal tissue in congenital adrenal hyperplasia (CAH) patients. The present manuscript is about an unusual case of a 16-year-old CAH patient due to 11β-hydroxylase deficiency. He underwent testicle biopsy because of testicle tumor suspicion and diagnosed with TART.
متن کاملCongenital adrenal hyperplasia and Leydig cell tumor of testis. Case report and review of literature.
Adrenogenital Syndrome, more properly defined as Congenital Adrenal Hyperplasia (CAH), is related to the enzyme 21-beta-hydroxylase deficiency, with impaired glucocorticoids and aldosterone syntheses and increased ACTH synthesis. This report describes a case of a monorchid patient suffering from Adrenogenital Syndrome and Leydig cell tumor of his testis. A right orchidectomy with implantation o...
متن کامل