ESPE Abstracts (2021) 94 P2-26

Putrajaya Hospital, Putrajaya, Malaysia


Introduction: Testicular adrenal rest tumour (TART) is a complication in males with congenital adrenal hyperplasia (CAH). However, the prevalence, risk factors and treatment in children are not clear.

Objective: 1) To identify the incidence of TART and risk factors in patients with classical CAH in our centre 2) To study the clinical characteristics, hormonal profile, treatment interventions and outcome of patients with TART.

Methods: Testicular ultrasound were performed in 16 patients with poorly controlled CAH (mean age 9.87 ± 3.11, range 4.67-16yrs). Clinical characteristics, hormonal profiles and treatment were compared between patients with and without TART.

Results: TART lesions were detected by ultrasound in 6 patients (37.5%). The lesions were bilateral in all patients, mean diameter of 0.3cm, hypoechoic in nature and scarce in vascularity. Interestingly, testicular microlithiasis was found coincidently in one patient. There was no significant difference in height SDS and BMI SDS in patients with and without TART. TARTs were detected in 60% of patients who had attained final height. Only 1 patient had palpable nodules. The 17-hydroxyprogesterone level was higher in CAH patients with TART (322.22 ± 238.87nmol/l) than in patients without TART (149.61 ± 201.20nmol/l). Poor compliance was identified in 5 patients. 5 patients developed peripheral precocious puberty and two patients evolved into central precocious puberty. All patients were on hydrocortisone and fludrocortisone replacement. All had intensified hydrocortisone regimen after detection of TART (mean dose 17.3mg/m2/day) for 2-4 weeks, however only two had resolution of TART on repeated ultrasound.

Table 1. Comparison of clinical characteristics of patients with and without TART
With TART (n = 6)Without TART (n = 10)p value
Age, years*9.6110.030.806
Weight SDS*1.290.290.150
Height SDS*0.76-0.240.213
BMI SDS*1.180.820.471
Bone age advancement*4.891.280.037
17OHP*322.22149.610.143
Hydrocortisone*15.1014.140.371
*the values were expressed as mean
Table 2. Clinical characteristics of patients with TART
PatientAge of CAH diagnosis, yearsAge of TART diagnosis, years17OHP (nmol/l)Hydrocortisone dose (mg/m2/day)Resolution of TART
10.094.67>60.618.0Yes
20.36.008.814.5Yes
30.0913.08141.618.0No
40.0913.83572.018.4No
53.508.33246.217.9No
60.0911.75614.916.9No

Conclusions: Ultrasound of testes should be routinely performed in childhood, especially in those with poor disease control and at peri-pubertal age. Larger studies should be conducted to explore the risk factors and therapeutic modalities in TART.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

Online,
22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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