Testicular adrenal rest tumours in congenital adrenal hyperplasia

Grinten, Hedi L Claahsen–van der; Otten, Barto J.; Stikkelbroeck, Monica; Sweep, Fred C.G.J.; Hermus, A.R.M.M.

doi:10.1016/j.beem.2008.09.007

Cited by 159 publications

(208 citation statements)

References 67 publications

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“…This is also in accordance with a study by Casteràs et al (29) which showed low fecundity in classic CAH patients but normal fertility in patients with parental project and spontaneous pregnancy in patients with strict control of progesterone concentrations in the follicular phase of the menstrual cycle. In men, TARTs were the only event that could not be linked to hormonal control, but they were associated with the severity of the disease (30,31,32). Indeed, TARTs were found only in patients with CF CAH.…”

Section: Discussionmentioning

confidence: 99%

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Bachelot¹,

Golmard²,

Jérôme³

et al. 2015

European Journal of Endocrinology

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Aim: Adverse outcomes in adult congenital adrenal hyperplasia (CAH) patients are frequent. The determinants of them have not yet been established. Objective: To establish the prevalence of adverse outcomes and to find determining factors for each of them. Design, patients, and methods: Cross-sectional monocentric study of 104 patients with childhood onset of CAH (71 women, 33 men). Analysis established first the determinants of clinical, hormonal, genetic variables and second a composite criterion for some of the outcomes and determinants. Results: BMI was above 25 kg/m 2 in 44% of the cohort, adrenal hyperplasia and/or nodules were present in 45% of the patients, and irregular menstrual cycles and hyperandrogenism were found in 50 and 35% of the women respectively. In univariate analysis, the determinants of these outcomes were all linked to disease control, especially 17-hydroxyprogesterone (17OHP) and androstenedione concentrations. Low weight was a determinant of abnormal bone mineral density (BMD) (60% of the cohort). Multivariate analysis confirmed these data. A classic form (CF) of CAH was a determinant of testicular adrenal rest tumors (TARTs) (36% of the men). Total cumulative glucocorticoid dose was a determinant of BMI and TART, whereas fludrocortisone dose was a determinant of TART (PZ0.03). In men, the composite criterion was associated with androstenedione concentration and CF. In women, the composite criterion was associated with total testosterone concentration. Conclusion:The present study confirms the high prevalence of adverse outcomes in CAH patients. These are, most often, related to disease control. The impaired health status of adults with CAH could therefore be improved through the modification of treatment.

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Section: Discussionmentioning

confidence: 99%

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Bachelot¹,

Golmard²,

Jérôme³

et al. 2015

European Journal of Endocrinology

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“…TARTs are thought to originate from aberrant adrenal cells descending during embryogenesis with testicular cells. ACTH-suppressive glucocorticoid treatment is widely believed to result in TART shrinkage [70]. However, in cross-sectional studies, ACTH concentrations and TART size did not correlate and TARTs even occurred in overtreated patients as indicated by suppressed ACTH concentrations [64,65,67].…”

Section: Male Fertilitymentioning

confidence: 99%

Health Problems in Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Reisch

Arlt

Krone³

2011

Horm Res Paediatr

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Following the introduction of life-saving glucocorticoid replacement 60 years ago, congenital adrenal hyperplasia (CAH) has evolved from being perceived as a paediatric disorder to being recognized as a lifelong, chronic condition affecting patients of all age groups. Increasing evidence suggests that patients with CAH have an increased risk to develop health problems during adult life, with signs and symptoms of forerunner conditions of adult disease already emerging during the time of paediatric care. Transition of paediatric CAH patients to medical care in the adult setting is an important step to ensure optimal lifelong treatment, aiming to achieve good health and normal life expectancy and quality of life. Thus, primary and secondary prevention of health problems has to become a task of increasing importance for those involved in the care of CAH patients throughout their life.

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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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Testicular adrenal rest tumours in congenital adrenal hyperplasia

Cited by 159 publications

References 67 publications

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Health Problems in Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

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

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