Testicular Adrenal Rest Tumor in 11-Beta-Hydroxylase Deficiency Driven Congenital Adrenal Hyperplasia
نویسندگان
چکیده
Testicular adrenal rest tumors (TART) occur often as asymptomatic nodules in corticotropin-dependent lesions aberrant adrenal tissue in congenital adrenal hyperplasia (CAH) patients. The present manuscript is about an unusual case of a 16-year-old CAH patient due to 11β-hydroxylase deficiency. He underwent testicle biopsy because of testicle tumor suspicion and diagnosed with TART.
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 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...
متن کاملTesticular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue.
CONTEXT In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors are frequently found that may interfere with gonadal function. OBJECTIVE Our objective was to determine steroid-producing features of testicular adrenal rest tumors. DESIGN AND SETTING The study is descriptive and took place at a university medical center. PATIENTS Eight adult CAH patients w...
متن کاملبررسی 10 ساله تظاهرات بالینی و شیوع انواع هیپرپلازی مادرزادی آدرنال در انستیتو غدد درون‌ریز و متابولیسم
Background & Objective: Congenital Adrenal Hyperplasia(CAH) includes a group of inherited diseases which are caused by enzyme defects in the synthesis of cortisol from cholesterol. It manifests itself in different forms like ambiguous genitalia, adrenal crisis in infants, precocious puberty in children, hirsutism, oligomenorrhea and infertility in adults. Although CAH is one of the most com...
متن کاملCONGENITAL ADRENAL HYPERPLASIA IN NORTH•EAST OF IRAN: A REVIEW OF 47 PATIENTS AND THE ROLE OF PARENTAL CONSANGUINITY IN THE OCCURRENCE OF DISEASE
In this study the clinical and epidemiological characteristics of congenital adrenal hyperplasia were evaluated prospectively in 47 patients admitted in Imam Reza Hospital in Mashhad during a 4 year period. 21-hydroxylase deficiency was present in 42 patients (89.3%), the simple virilizing form in 6 and the salt-losing form in 36 of them. 11b hydroxylase deficiency was present in 5 patient...
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