Gonadal and adrenal catheterization during adrenal suppression and gonadal stimulation in a patient with bilateral testicular tumors and congenital adrenal hyperplasia.

Combes-Moukhovsky, M E; Kottler, Marie Laure; Valensi, P.; Boudou, P.; Sibony, Mathilde; Attali, J. R.

doi:10.1210/jcem.79.5.7962333

Cited by 25 publications

(9 citation statements)

References 20 publications

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“…When no testicular biopsy is available, however, some characteristics, such as the bilateral presence of lesions, the location of the lesions adjacent to the mediastinum and the decrease in size to glucocorticoid therapy, can be helpful in differentiating between a (benign) adrenal rest tumour and a malignant testicular lesion [12,17,21]. Also, steroid measurements in serum from testicular vein sampling can indicate the presence of active adrenal rest tissue in the testis [12,22,23].…”

Section: Discussionmentioning

confidence: 99%

Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features

et al. 2003

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The purpose of this study was to investigate the prevalence of testicular adrenal rest tumours in patients with congenital adrenal hyperplasia (CAH), and to describe sonographic and MR features of these lesions. Seventeen postpubertal male CAH patients underwent scrotal sonography, with colour Doppler, and in 16 of them pre- and postcontrast enhanced T1- and T2-weighted MR images of the testes were obtained. Ultrasound revealed lesions in 16 of 17 patients (94%), bilateral in 10 patients and unilateral in 6 patients. The lesions were typically located adjacent to the mediastinum testis. The maximal diameter of the lesions varied from 2 to 40 mm. Margins were blurred in 11 of 31 lesions. Seventeen of the 20 lesions smaller than 2 cm in diameter were hypoechoic, whereas all 11 lesions larger than 2 cm showed hyperechoic reflections. On MR all lesions were isointense on T1- and hypointense on T2-weighted images and lesion margins were clearly defined. Enhancement of the lesions after intravenous contrast was seen in 13 of 15 patients. In our series the prevalence of testicular adrenal rest tumours in postpubertal CAH patients is much higher than in other reported studies. The lesions may develop from some small, hypoechoic, and multifocal nodules and coalesce to large hypoechoic lesions with hyperechoic reflections on ultrasound. As our results suggest that ultrasonography and MR show the lesions equally well, ultrasonography should be the method of first choice for detection and follow-up of these lesions, because it is the cheapest and quickest imaging technique. In case of a partial orchiectomy, MR is recommended because it shows lesion margins optimally.

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Section: Discussionmentioning

confidence: 99%

Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features

et al. 2003

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“…Based on histological studies, TARTs have close resemblance to adrenocortical tissue [130,131]. Functional studies in patients with 21OHD have identified ACTH and angiotensin II (AII) receptors and expression of CYP11B1 and CYP11B2 adrenal specific enzymes [132][133][134][135][136]. Presence of ACTH and AII receptors in the TART tissue indicates hormone responsive tissue which can undergo hypertrophy and/or hyperplasia due to elevated ACTH (and/ or AII) levels [132,137,138].…”

Section: Testicular Adrenal Rest Tumorsmentioning

confidence: 99%

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

2016

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Congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is a rare autosomal recessive genetic disorder. It is caused by reduced or absent activity of 11β-hydroxylase (CYP11B1) enzyme and the resultant defects in adrenal steroidogenesis. The most common clinical features of 11 beta-hydroxylase deficiency are ambiguous genitalia, accelerated skeletal maturation and resultant short stature, peripheral precocious puberty and hyporeninemic hypokalemic hypertension. The biochemical diagnosis is based on raised serum 11-deoxycortisol and 11-deoxycorticosterone levels together with increased adrenal androgens. More than 100 mutations in CYP11B1 gene have been reported to date. The level of in-vivo activity of CYP11B1 relates to the degree of severity of 11 beta-hydroxylase deficiency. Clinical management of 11 beta-hydroxylase deficiency can pose a challenge to maintain adequate glucocorticoid dosing to suppress adrenal androgen excess while avoiding glucocorticoid-induced side effects. The long-term outcomes of clinical and surgical management are not well studied. This review article aims to collate the current available data about 11 beta-hydroxylase deficiency and its management.

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“…One of the most important and frequently detected complications and the leading cause of infertility in adult male CAH patients is the development of benign testicular tumours [5,6,7]. As a result of the morphological and functional resemblance with adrenal tissue they are usually referred to as ‘testicular adrenal rest tumours' (TART) [8,9,10,11,12]. …”

Section: Introductionmentioning

confidence: 99%

Increased Prevalence of Testicular Adrenal Rest Tumours during Adolescence in Congenital Adrenal Hyperplasia

Grinten

Dehzad

Ulzen³

et al. 2014

Horm Res Paediatr

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Testicular adrenal rest tumours (TART) are one of the most important causes of infertility in adult male patients with congenital adrenal hyperplasia (CAH). These benign tumours are already detected in children, but screening of TART is not routinely performed. Objective: To define retrospectively the prevalence of TART in 41 paediatric male CAH patients aged 0-19 years regularly followed by high-frequency (F_central 12-MHz) ultrasound techniques. Results: Above the age of 10 years, there was a clear increase in the prevalence of TART: 10-12 years, 28% (2 of 7 patients), 13-14 years, 50% (4/8), and 15-16 years, 75% (3/4). Above the age of 16 years, TART were detected in 100% of the patients (7/7). The tumours were not detectable by palpation. Conclusion: TART is already present in childhood with an increasing prevalence after onset of puberty. We recommend regular ultrasound from the onset of puberty in all boys with classic CAH.

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Gonadal and adrenal catheterization during adrenal suppression and gonadal stimulation in a patient with bilateral testicular tumors and congenital adrenal hyperplasia.

Cited by 25 publications

References 20 publications

Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features

Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

Increased Prevalence of Testicular Adrenal Rest Tumours during Adolescence in Congenital Adrenal Hyperplasia

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