Testicular Tumors in Congenital Adrenal Hyperplasia: Steroid Measurements from Adrenal and Spermatic Veins

Blumberg-Tick, J.; Boudou, P.; Nahoul, K; Schaison, G

doi:10.1210/jcem-73-5-1129

Cited by 58 publications

(27 citation statements)

References 27 publications

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“…Usually it refers to lack of regression or to recurrence after excision, even though steroidogenesis had been suppressed. Impressive cases of TAR autonomization from ACTH are the one reported by Blumberg-Tick et al (8) and especially the one reported by Clark et al (5). At 19 years of age, TAR responded to corticosteroids within one week, but 4 years later -with serum FSH at 21 mU/l and LH at 11 mU/l -TAR were unresponsive to 4 mg/day dexamethasone, even though serum 17-OH P was suppressed (5).…”

Section: Discussionmentioning

confidence: 99%

Testicular adrenal rests: evidence for luteinizing hormone receptors and for distinct types of testicular nodules differing for their autonomization

Benvenga

Smedile²,

Giudice³

et al. 1999

European Journal of Endocrinology

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We report one patient with 21-hydroxylase deficiency and associated bilateral macro-orchidism caused by nodular hyperplasia of testicular adrenal rests (TAR). The boy, referred to us when 10 years old, was born with bilateral cryptorchidism that was treated unsuccessfully with i.m. injections of human chorionic gonadotropin (hCG) and later on with orchidopexy. He was treated with oral dexamethasone (0.625 mg per day) for the following 13 years.After one year, there was a marked reduction in steroid hormone levels (17-hydroxyprogesterone (17-OH P) from 27.2 to 1.2 nmol/l, testosterone from >104 to 4.8 nmol/l, estradiol (E 2 ) from 481 to 33 pmol/l). After the same period of time, both testicular volume and nodularity decreased: from 45 to 18 ml and from numerous to four nodules in the right testis, and from 40 to 13 ml and from numerous to three nodules in the left testis. At the third year, there were transient increases in serum gonadotropins, testicular volume (right testis = 25 ml, left testis = 20 ml) and steroid hormones, including cortisol (serum ACTH and dehydroepiandrosterone sulfate remained suppressed). At the fourth year of follow-up, there were still four nodules in the right testis and three in the left testis. The LH-dependency (which implies possession of LH/hCG receptors) of these nodules was also substantiated by their steroidogenic response to an acute i.m. hCG test. An exogenous ACTH stimulation test increased serum 17-OH P and cortisol. Since these nodules, unlike the majority of those present initially, were not suppressed by the corticosteroid therapy and since they were not detected when the patient returned for control at 23 years of age, they had partial autonomy from ACTH. At 23 years of age, the patient had a single nodule in the right testis (right testis volume = 13 ml, left testis volume = 10 ml), which should have accounted for the consistent difference in size between the two gonads. Serum LH was about 7 mU/l and FSH about 23 mU/l. The responsiveness of plasma steroid hormones to hCG had changed quantitatively and qualitatively. Secretion of cortisol was absent, secretion of 17-OH P and testosterone was reduced, and secretion of E 2 was much increased. The ACTH stimulation test showed that serum cortisol did not respond, while the other steroids responded in the order of 17-OH P > E 2 > testosterone.We conclude that there were three different groups of TAR when the patient was already 10 years old: (i) ACTH-sensitive (the majority), (ii) partially ACTH-insensitive but LH/hCG-sensitive (three nodules in the left testis and three in the right testis), (iii) almost entirely ACTH-insensitive and partially hCG-insensitive (a single nodule in the right testis). Probably, the never suppressed gonadotropin levels (presumably due to the bilateral testicular damage subsequent to the cryptorchid state) and the hCG therapy were major etiological factors for the appearance of the second and third population of TAR.

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Section: Discussionmentioning

confidence: 99%

Testicular adrenal rests: evidence for luteinizing hormone receptors and for distinct types of testicular nodules differing for their autonomization

Benvenga

Smedile²,

Giudice³

et al. 1999

European Journal of Endocrinology

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“…5 The extra-adrenal expression of steroid 21-hydroxylase and 11␤-hydroxylase by a benign testicular Leydig cell tumor has also been reported. 6 We reported that aldosterone, synthesized in the vasculature, is in part controlled by angiotensin (Ang) II and participates in the development of vascular hypertrophy that correlates with Ang II concentration. 7,8 We performed the present study to determine whether the rat heart produces aldosterone and to investigate the effects of adrenalectomy, ACE inhibitor, and Ang II on aldosterone synthesis, aldosterone synthase activity, and the expression of aldosterone synthase gene (CYP11B2) in the heart.…”

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confidence: 99%

Cardiac Aldosterone Production in Genetically Hypertensive Rats

et al. 2000

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Abstract-Aldosterone is synthesized in extra-adrenal tissues, both blood vessels and brain. We undertook the present study to determine whether the rat heart produces aldosterone and to investigate the effects of adrenalectomy, ACE inhibition, and angiotensin II on aldosterone synthesis in the heart. To clarify the pathophysiological role of cardiac aldosterone in the hypertensive heart, we compared the synthesis of aldosterone in the hearts of stroke-prone spontaneously hypertensive rats (SHRSP) with that in Wistar-Kyoto rats. The effects of the aldosterone antagonist spironolactone on myocardial hypertrophy in adrenalectomized SHRSP were also studied. Isolated rat hearts were perfused for 2 hours, and the perfusate was analyzed with HPLC and mass spectrometry. The activity of aldosterone synthase was estimated on the basis of the conversion of [ 14 C]deoxycorticosterone to [ 14 C]aldosterone. The levels of aldosterone synthase gene (CYP11B2) mRNA were determined with competitive polymerase chain reaction. Aldosterone production, the activity of aldosterone synthase, and the expression of CYP11B2 mRNA were increased in hearts from adrenalectomized rats and rats treated with angiotensin II. ACE inhibitors decreased cardiac aldosterone synthesis. Cardiac aldosterone, aldosterone synthase activity, and CYP11B2 mRNA levels in hearts from 2-and 4-week-old SHRSP were significantly greater than those of age-matched Wistar-Kyoto rats. Spironolactone prevented cardiac hypertrophy in adrenalectomized SHRSP. These results suggest that the rat heart produces aldosterone and that endogenous cardiac aldosterone may affect cardiac function and hypertrophy in hypertension in rats. (Hypertension. 2000;36:495-500.)Key Words: heart Ⅲ rats, inbred strains Ⅲ aldosterone Ⅲ hypertrophy Ⅲ cytochrome p450 Ⅲ mRNA A ldosterone receptors are present in cardiac myocytes, endocardial endothelial cells, and cardiac fibroblasts. 1 The peripheral infusion of aldosterone in rats causes cardiac hypertrophy and cardiac fibrosis without increasing the blood pressure. 2 In cultured neonatal rat fibroblasts or cardiomyocytes, aldosterone increases collagen synthesis and protein synthesis. 3,4 Physiologically significant amounts of mineralocorticoids are believed to be synthesized only in the adrenal cortex. However, extra-adrenal steroid 21-hydroxylation and 11␤-hydroxylation occur in a variety of human tissues. Circulating progesterone is converted to deoxycorticosterone (DOC) and DOC sulfate in adult and fetal human extra-adrenal tissues. 5 The extra-adrenal expression of steroid 21-hydroxylase and 11␤-hydroxylase by a benign testicular Leydig cell tumor has also been reported. 6 We reported that aldosterone, synthesized in the vasculature, is in part controlled by angiotensin (Ang) II and participates in the development of vascular hypertrophy that correlates with Ang II concentration. 7,8 We performed the present study to determine whether the rat heart produces aldosterone and to investigate the effects of adrenalectomy, ACE inhibitor, and Ang ...

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“…This paradoxical result could suggest poor compliance or secretion of androstenedione by the testicular tumour itself. In that case, the androstenedione level is not only the result of adrenal activity but also of tumour activity and cannot be used to assess adrenal suppression 15 ' 16 .…”

Section: Discussionmentioning

confidence: 99%

“…In one patient (#2), the regimen of hydrocortisone 2 times a day (15 + 10 mg) was changed into dexamethasone 3 times a day (0.25 + 0.25 + 0.25 mg), to improve semen quality after oligoasthenoteratozoospermia was found and he and his wife wanted to have a child. In seven patients (#5, 6,8,12,15,16,17), the hydrocortisone regimen (3 times a day) had been changed into hydrocortisone in the morning (and afternoon) and dexamethasone in the evening. Changes in body mass index between baseline and follow-up investigation did not show a significant correlation with changes in glucocorticoid dose (Spearman rank,…”

Section: Glucocorticoid Therapy Between Baseline and Followup Investimentioning

confidence: 99%

Asymptomatic Testicular Adrenal Rest Tumours in Adolescent and Adult Males with Congenital Adrenal Hyperplasia: Basal and Follow-up Investigation After 2.6 Years

Stikkelbroeck¹,

Hermus²,

Suliman³

et al. 2004

Journal of Pediatric Endocrinology and Metabolism

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Aim:To study the course of asymptomatic testicular adrenal rest tumours in patients with congenital adrenal hyperplasia (CAH) and the association between tumour changes and glucocorticoid therapy adjustments. Patients and Methods: Fifteen male patients with CAH (21-hydroxylase deficiency), in whom asymptomatic testicular adrenal rest tumours had been found at a baseline investigation, underwent scrotal ultrasonography and venous blood sampling (for LH, FSH and testosterone) on average 2.6 years later. The level of hormonal control was assessed by measurement of androstenedione in three diurnal saliva samples. Data on changes in glucocorticoid therapy since baseline were obtained from the patients' records. Results: Tumour decrease, defined as >30% decrease in the sum of the longest diameter(s) of the lesion(s), was found in six patients; tumour increase, defined as >20% increase, in six and stable tumours in three patients. All three patients with overtreatment showed tumour decrease and of the six patients with undertreatment only one showed tumour decrease. Tumour increase was not only observed in undertreated patients but also in patients with adequate treatment. Changing the night dose of hydrocortisone into dexamethasone, to obtain Conclusions: Tumour decrease could be achieved by aiming at adrenal oversuppression, but the required high glucocorticoid doses may induce side effects. In asymptomatic tumours in young male patients with CAH, a practical guideline could be to optimise adrenal suppression to a maximal tolerable glucocorticoid dose and to offer analysis and cryopreservation of semen as soon as the patient can be motivated.

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Testicular Tumors in Congenital Adrenal Hyperplasia: Steroid Measurements from Adrenal and Spermatic Veins

Cited by 58 publications

References 27 publications

Testicular adrenal rests: evidence for luteinizing hormone receptors and for distinct types of testicular nodules differing for their autonomization

Testicular adrenal rests: evidence for luteinizing hormone receptors and for distinct types of testicular nodules differing for their autonomization

Cardiac Aldosterone Production in Genetically Hypertensive Rats

Asymptomatic Testicular Adrenal Rest Tumours in Adolescent and Adult Males with Congenital Adrenal Hyperplasia: Basal and Follow-up Investigation After 2.6 Years

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