Testicular Adrenal Rest Tumors in Patients with Congenital Adrenal Hyperplasia

Abstract

Objective: Early diagnosis and treatment of testicular adrenal rest tumors (TART) is important for gonadal functions and fertility protection in boys with congenital adrenal hyperplasia (CAH). In this descriptive study, we investigated the prevalence of TART in boys with 21-hydroxylase deficient (21OHD) CAH followed in our pediatric endocrine clinic.Methods: The study group consisted of 14 male patients with a mean age of 9.6±5.1 (range: 0.8-18.3) years. Six (42.9%) of the 14 patients were diagnosed as having salt-wasting type (SW) and eight (57.1%) patients - as having the simple virilizing (SV) form of 21OHD. Mean age at diagnosis was 2.9±2.7 (range: 0.03-6.3) years. Two different radiologists performed scrotal ultrasonography. Chronological age, bone age, and anthropometric measurements were evaluated. Serum adrenocorticotropic hormone (ACTH), 17-alpha-hydroxyprogesterone (17OHP) and androstenedione levels were also evaluated in all patients during the follow-up period.Results: Scrotal ultrasonography revealed bilateral TART in two patients (14.3%) and testicular microlithiasis (TM) in four patients (28.6%). One patient had both TART and TM bilaterally. During the follow-up period, the mean serum adrenocorticotropic hormone, 17OHP and androstenedione levels in the total group of patients were 130.0±179.1 pg/mL (21.7-726.5), 5.8±3.3 ng/mL (0.8-11.4) and 4.3±4.1 (0.2-11.0) ng/mL, respectively.Conclusions: Microlithiasis or TART may be frequently encountered during the follow-up of patients with CAH. In order to prevent late complications including infertility, we suggest that ultrasonographic evaluations be performed yearly in all male CAH patients. Conflict of interest:None declared.

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

“…The presence of TART in children is described mostly in case reports and only a limited number of studies describe its prevalence in larger populations of children and adults [23,24,25,26]. It is suggested that adrenal rest cells in the testes in CAH patients are already present in early infancy [27], but the onset of tumour growth is not known.…”

Testicular Adrenal Rests Tumors and Testicular Microlithiasis in a Brazilian Case Series with Classic Congenital Adrenal Hyperplasia

“…It was more representative in a group in the final height (group 2). Other authors ( 3 , 9 , 11 , 12 , 15 , 20 , 21 ) had related a positive correlation between age, pubertal stage, and presence of TART, with the highest prevalence in adults ( 22 ), up to 94% ( 7 , 8 ). All these findings reinforce hypothesis of LH elevation as an important factor in development of TART ( 9 , 10 ), which are present since the intrauterine period, but are more prevalent in adulthood ( 2 ).…”

“…It was defined as good control of CAH when serum level of 17-hydroxyprogesterone (17OHP) ≤ 10 ng/mL and poor control when above this value ( 15 , 16 ). The control was considered suppressed when androstenedione (Δ4) levels are below normal for their age ( 9 ) (children: < 0.3 ng/mL and men < 0.4 ng/mL).…”

The state of the Sertoli cells in the children and adolescents presenting with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

“…Частота ТАРТ у мужчин с ВДКН зависит от ко-горты пациентов, метода выявления опухоли, и ва-рьирует от 0 до 94%. Опухоли у детей и подростков с ВДКН выявляются в 20-40% случаев [19,[21][22][23][24]. В нашем исследовании частота TART оказалась не-сколько ниже, что, по-видимому, связано с возрас-том пациентов.…”