Large testicular adrenal rest tumours in a patient with congenital adrenal hyperplasia
نویسندگان
چکیده
UNLABELLED Testicular adrenal rest tumours (TARTs) are benign ACTH-dependent tumours that occur in males with congenital adrenal hyperplasia (CAH) and if left untreated can destroy testicular tissue. Corticosteroid suppressive treatment could result in the regression of these testicular tumours. We present a patient with bilateral large TARTs as a consequence of poor compliance to treatment and follow-up for his CAH, who consequently had to have bilateral orchidectomies and prosthesis replacement. LEARNING POINTS TARTs are frequently seen in males with CAH, and can be misdiagnosed as primary testicular cancer.Patient compliance to treatment and follow-up are necessary to reduce the risk of testicular damage as a result of TARTs in patients with CAH.Boys with CAH should have periodic ultrasonographic screening from before adolescent age for early detection of TARTs.Regular monitoring of renin, 17-hydroxyprogesterone and androgens levels is required to assess corticosteroid suppressive treatment.Patients with CAH should be offered psychological support and information concerning CAH support groups.
منابع مشابه
A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
21-hydroxylase deficiency (21-OHD) caused congenital adrenal hyperplasia (CAH) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO) synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs) are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The p...
متن کاملTesticular Adrenal Rest Tumours in Congenital Adrenal Hyperplasia
In adult patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumours (TART) is an important complication leading to gonadal dysfunction and infertility. These tumours can be already found in childhood and puberty. In this paper, we review the embryological, histological, biochemical, and clinical features of TART and discuss treatment options.
متن کاملAn adrenal rest tumour in the perirenal region in a patient with congenital adrenal hyperplasia due to congenital 3beta-hydroxysteroid dehydrogenase deficiency.
BACKGROUND In contrast to the high incidence of testicular adrenal rest tumours (TART) in adult male patients with congenital adrenal hyperplasia (CAH), ovarian adrenal rest tumours in female CAH patients are very rare and other locations of adrenal rest tumours have never been reported. Here, we report on an adult patient with CAH due to 3beta-hydroxysteroid dehydrogenase (HSD) deficiency with...
متن کاملAsymptomatic testicular adrenal rest tumours in adolescent and adult males with congenital adrenal hyperplasia: basal and follow-up investigation after 2.6 years.
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, underwen...
متن کاملتومور دو طرفه بیضه همراه با هیپرپلازی مادرزادی آدرنال و کمبود آنزیم 11-هیدروکسیلاز از بیمارستان امام
The development of testicular masses in male patients with congenital adrenal hyperplasia due to 21 hydroxylase deficiency has been recognized for many years. We present here the eighth and ninth reported patient with bilateral testicular tumors associated with 11 hydroxylase deficiency. They were two brothers aged 7.5 and 5 yr. who had bilateral testicular tumors and diagnosed because of signs...
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ورودعنوان ژورنال:
دوره 2015 شماره
صفحات -
تاریخ انتشار 2015