1/15. Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report.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.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
2/15. Bilateral testicular adrenal rest tissue in congenital adrenal hyperplasia: US and MR features.We describe magnetic resonance (MR) and ultrasonography (US) features of bilateral testicular adrenal rest tissue in a 20-yr-old man with congenital adrenal hyperplasia (CAH). Scrotal ultrasonology detected bilateral homogeneous hypoechoic lesions with well-defined margins and without evidence of sound attenuation. MR is useful in defining the size of lesions, because the contrast resolution is better than with sonography and allows an accurate definition of the extent of disease. This case suggests that US evaluation should be included in periodical follow-up of patients with CAH, while MR may be used in the case of rapid increase in the size of the testicular mass.- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
3/15. Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo and in vitro studies).We describe the case of a 20-year-old patient with salt-wasting congenital adrenal hyperplasia (CAH) related to 21-hydroxylase deficiency. Bilateral craggy testicular tumours were found, requiring histological evaluation. Prior to the surgical procedure, the patient was treated with dexamethasone (he presented cortisol deficiency) and was stimulated with ACTH. High levels of 11beta-OH steroids measured in the gonadal vein, compared with peripheral blood samples suggested the presence of adrenal rests. Incubation of the tumours (which could not be differentiated histologically, from Leydig tissue), with radioactive steroid precursors was carried out. The results revealed the testicular tumours were of adrenal tissue origin, associated with 21-hydroxylase deficiency. The patient's non-compliance to glucocorticoid treatment was the main cause of his hypogonadotropic hypogonadism.- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
4/15. testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia.The association between testicular tumors/nodules and congenital adrenal hyperplasia (CAH) has been recognized for many years. Tumors are considered to be an aberrant adrenal tissue that has descended with the testes and has become hyperplastic due to ACTH stimulation. The recommended treatment consists of increasing the glucocorticoid dose to suppress ACTH secretions. If the testicular size is not reduced after suppression therapy or a side effect of glucocorticoid dose is noted, surgical intervention should be considered. We diagnosed steroid unresponsive testicular tumors of the CAH in two patients who were treated by testicular sparing tumor enucleation. We believe that testis sparing surgery is the procedure of choice for all patients with testicular adrenal rest tumor, since it maximizes future fertility potential.- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
5/15. Ovarian adrenal rest tissue in congenital adrenal hyperplasia--a patient report.We report a young girl who died in an Addisonian crisis due to previously undiagnosed congenital adrenal hyperplasia (CAH), in whom ovarian adrenal rest tissue was detected at postmortem histopathological examination. This is a very rare complication in female patients with CAH with only two previously reported cases.- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
6/15. Testicular adrenal rest hyperplasia due to 21-hydroxylase deficiency: a case report.Bilateral testicular tumors are a rare complication of congenital adrenal hyperplasia. It can be extremely difficult to distinguish histologically between Leydig cell tumors and adrenocortical rest hyperplasia, which may lead in some cases to unnecessary orchidectomy. A 7-yr-old boy was admitted because of precocious puberty and enlargement of testicles. Hormonal studies established the diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Testicular biopsy revealed interlacing strands, cords, and rests of cells resembling interstitial (Leydig) cells but with no Reinke crystalloids. Here we report a case of testicular adrenal rest hyperplasia in congenital adrenal hyperplasia and discuss the pathological and clinical features and origin of this rare lesion by using immunohistochemical evaluation.- - - - - - - - - - ranking = 9keywords = hyperplasia (Clic here for more details about this article) |
7/15. Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia.Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed golgi apparatus, and mitochondria with predominantly tubular cristae, features characteristic of steroid-secreting cells of adrenocortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 micrograms/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin. The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.- - - - - - - - - - ranking = 6keywords = hyperplasia (Clic here for more details about this article) |
8/15. Bilateral testicular tumours in congenital adrenal hyperplasia: a continuing diagnostic and therapeutic dilemma.Bilateral testicular tumours are a rare but well described complication of congenital adrenal hyperplasia (CAH). It can be extremely difficult to distinguish histologically between Leydig cell tumours and adrenocortical rest hyperplasia which may lead in some cases to unnecessary orchidectomy in young men. A 26-year-old male in whom CAH had been diagnosed neonatally, was referred for further investigation of a craggy mass in the left testis. Six months earlier, right orchidectomy had been performed after presentation with bilateral enlarged, hard testes and azoospermia. The tumour was interpreted to be a malignant interstitial cell tumour of the testis. Our investigations revealed that his CAH was inadequately controlled and that the hormonal secretion from the remaining left testicular mass was ACTH dependent. Percutaneous venous sampling revealed high concentrations of cortisol and other adrenocortical hormones coming from the gonadal vein. dexamethasone suppressed these adrenal hormones and reduced the size and softened the consistency of the testicular mass. Total disappearance of the mass was achieved by using dexamethasone, given in a reverse circadian rhythm regimen. spermatogenesis returned and the patient's wife became pregnant after 6 months; she gave birth to a normal male infant. To our knowledge, this is the first time in this clinical setting that fertility has been achieved. This case highlights the need for both a functional and histological assessment of such 'tumours' in CAH prior to orchidectomy.- - - - - - - - - - ranking = 6keywords = hyperplasia (Clic here for more details about this article) |
9/15. Lipid cell tumor of the ovary in reference to adult-onset congenital adrenal hyperplasia and polycystic ovary syndrome. A case report.A 30-year-old woman with a virilizing lipid tumor was initially suspected of having adult-onset congenital adrenal hyperplasia (CAH) when her plasma 17-hydroxyprogesterone (17OHP) concentration was found to be very high (298-3,170 ng/dL), to drop in response to an overnight dexamethasone (dex) suppression test (79 ng/dL) and to rise briskly 15 minutes after ACTH administration (751 ng/dL). However, the effect of dex was not sustained or complete: the pregnanetriol excretion dropped only from 5.5 to 4.4 mg daily. Furthermore, the plasma testosterone was inappropriately high (235-537 ng/dL) for adult-onset CAH and was more responsive to endogenous and exogenous gonadotropin stimulation than to ACTH. In addition, there was no evidence of 11 beta-hydroxylation of 21-deoxycorticoids, as would be expected in CAH. Removal of the tumor completely reversed the virilization and the abnormal responses to ACTH and human chorionic gonadotropin. The contralateral ovary bore lipid-laden stromal cells in the deep paracortex that bore a striking resemblance to the tumor cells, and a cyst in that ovary had fluid with a steroid pattern virtually identical to that of the tumor, with an androstenedione: 17OHP ratio of 5:1. There was not evidence of polycystic ovary disease. The clinical picture of type II polycystic ovary syndrome (PCOS) gradually evolved over a one-year period postoperatively: plasma-free testosterone became mildly elevated and was not dex suppressible. The 17OHP response to ACTH became slightly excessive. However, there was no evidence of tumor on computed tomography.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
10/15. Intratesticular adrenal rests diagnosed by ultrasound.Adrenal rest tumors of the testes may occur in conditions associated with increased circulating adrenocorticotropic hormone (ACTH), including congenital adrenal hyperplasia (CAH) and addison disease. Sonographically, these tumors appear as multiple round, hypoechoic nodules near the testicular hilus and are usually bilateral. They may undergo extensive fibrosis and become hyperechoic with acoustic shadowing. In the absence of excess ACTH or characteristic ultrasound findings, biopsy is recommended to exclude malignancy. Because malignant degeneration is very rare, close clinical and sonographic follow-up without biopsy is generally sufficient. Serial sonograms are useful to document stability or regression of tumor size during glucocorticoid replacement therapy.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
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