Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report.
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Congenital adrenal hyperplasia (CAH) is a well-recognized, but uncommon, cause of azoospermia and infertility in men. Commonly this is due to undertreatment of excessive adrenal androgen secretion which suppresses gonadotrophin stimulation of the testes. A less common complication of CAH is development of adrenal tissue within the testes; this is important to recognize because it may be confused with malignancy leading to unnecessary surgery. In this case report, a man is described with simple virilizing CAH due to 21-hydroxylase deficiency who presented with azoospermia and was found to have adrenal rests. Investigations concluded that there was adequate adrenal suppression with glucocorticoids and that azoospermia was due to obstruction by adrenal rest tissue, strategically situated at the hilum of the testes. Spermatozoa were able to be retrieved by testicular aspiration from the man and these were used to successfully establish a pregnancy using intracytoplasmic sperm injection of his wife's oocytes. (+info)
Nonfunctioning adrenocortical carcinoma.
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The rarity of adrenocortical carcinoma prompted us to report a case who came with a history of swelling in the left flank associated with pain, weakness and loss of appetite. Ultrasonography revealed a left retroperitoneal mass which was removed by radical surgery along with the left kidney and spleen. On histopathological examination, a diagnosis of adrenocortical carcinoma was made. (Hough criteria score 2.97). The cells of the tumor were arranged in closely packed columns and cords supported by fibrovascular stroma. There was no evidence of metastasis. (+info)
Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
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OBJECTIVE: Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction of the seminiferous tubules. The aim of our study was to determine the presence of TART and their influence on gonadal function in childhood. DESIGN: Retrospective study. PATIENTS AND METHODS: Scrotal ultrasound was performed in 34 children with CAH due to 21-hydroxylase deficiency who were between 2 and 18 years old. FSH, LH, testosterone and inhibin B concentrations were measured in serum of 27 patients. RESULTS: TART were detected by ultrasound in 8 out of 34 (24%) children. In two of them, bilateral tumours were found. All lesions were located in the rete testis. Seven patients had the salt-wasting type of CAH; one patient had the simple virilising type of CAH. Mean tumour size was 4.1 mm (range 2-8 mm). In none of the patients were the tumours palpable. Two children with TART were between 5 and 10 years old, the other six children were above 10 years old. In all children with TART, LH, FSH, testosterone and inhibin B levels were similar to the patients without TART. CONCLUSION: TART can be found in CAH children before the age of 10 years. The absence of gonadal dysfunction in our group of children suggests that gonadal dysfunction as frequently reported in adult CAH patients with TART develops after childhood. (+info)
An adrenal rest tumour in the perirenal region in a patient with congenital adrenal hyperplasia due to congenital 3beta-hydroxysteroid dehydrogenase deficiency.
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The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency.
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Development of a testicular adrenal rest tumor (TART) is common in males with congenital adrenal hyperplasia, and it can be an important cause of infertility. In the present study, we observed the prevalence of TARTs, and analyzed its associated factors in patients with 21-hydroxylase deficiency. Testicular ultrasonography was performed in 48 postpubertal male patients aged 10.6 to 27.1 years. To determine whether patients were undertreated, we analyzed the serum 17-hydroxyprogesterone (17-OHP) levels to the time of ultrasonographic measurement and calculated the percentage of measurements when serum 17-OHP level was >10 ng/mL relative to the total number of measurements during the follow-up period. We divided the 6-year period before ultrasonographic measurement (time 0) into three 2-year intervals and calculated the average concentration of serum 17-OHP in each interval to give a -2(nd) to 0 year-average concentration (-2-0YAC), -4-2YAC and -6-4YAC. A TART was detected by ultrasonography in 31 of 48 patients (64.6%) and the median maximal cross-sectional area of the TARTs was 0.71 (0.03, 4.95) cm(2). The corrected final adult height was lower, and -4-2YAC and body mass index were higher in patients with TART than in those without. After controlling for the type of 21-hydroxylase deficiency, hydrocortisone-equivalent dose, age, and -6-4YAC, the size of TART was associated with a high undertreatment percentage with a marginal statistical significance. These results suggest that strict disease control is mandatory and regular examination with testicular ultrasonography is recommended in male patients, regardless of the type of 21-hydroxylase deficiency. (+info)
Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review.
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Testicular adrenal rest tumours in boys with congenital adrenal hyperplasia: case report and literature review.
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Nodular testicular lesions derived from adrenal tissue (testicular adrenal rest tumours - TART) in boys and men with congenital adrenal hyperplasia (CAH) lead to testicular structure damage, spermatogenesis disorders, and infertility. Hyperplasia of the ectopic adrenal tissue in testes is associated with high levels of the adrenocorticotropic hormone (ACTH) in blood serum. The development of non-invasive methods of diagnostic imaging allows detection of testicular lesions in adolescents and children. The basic method for TART detection is imaging with ultrasonography (USG) being the most widely available method. Since these mild testicular lesions can cause impaired fertility, periodic palpation and testicular ultrasonography should be performed in patients with CAH in order to prevent infertility. (+info)