Cases reported "Cryptorchidism"

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1/60. Testicular adrenal rests: evidence for luteinizing hormone receptors and for distinct types of testicular nodules differing for their autonomization.

    We report one patient with 21-hydroxylase deficiency and associated bilateral macro-orchidism caused by nodular hyperplasia of testicular adrenal rests (TAR). The boy, referred to us when 10 years old, was born with bilateral cryptorchidism that was treated unsuccessfully with i.m. injections of human chorionic gonadotropin (hCG) and later on with orchidopexy. He was treated with oral dexamethasone (0.625 mg per day) for the following 13 years. After one year, there was a marked reduction in steroid hormone levels (17-hydroxyprogesterone (17-OH P) from 27.2 to 1.2 nmol/l, testosterone from >104 to 4.8 nmol/l, estradiol (E(2)) from 481 to 33 pmol/l). After the same period of time, both testicular volume and nodularity decreased: from 45 to 18 ml and from numerous to four nodules in the right testis, and from 40 to 13 ml and from numerous to three nodules in the left testis. At the third year, there were transient increases in serum gonadotropins, testicular volume (right testis = 25 ml, left testis = 20 ml) and steroid hormones, including cortisol (serum ACTH and dehydroepiandrosterone sulfate remained suppressed). At the fourth year of follow-up, there were still four nodules in the right testis and three in the left testis. The LH-dependency (which implies possession of LH/hCG receptors) of these nodules was also substantiated by their steroidogenic response to an acute i.m. hCG test. An exogenous ACTH stimulation test increased serum 17-OH P and cortisol. Since these nodules, unlike the majority of those present initially, were not suppressed by the corticosteroid therapy and since they were not detected when the patient returned for control at 23 years of age, they had partial autonomy from ACTH. At 23 years of age, the patient had a single nodule in the right testis (right testis volume = 13 ml, left testis volume = 10 ml), which should have accounted for the consistent difference in size between the two gonads. serum LH was about 7 mU/l and FSH about 23 mU/l. The responsiveness of plasma steroid hormones to hCG had changed quantitatively and qualitatively. Secretion of cortisol was absent, secretion of 17-OH P and testosterone was reduced, and secretion of E(2) was much increased. The ACTH stimulation test showed that serum cortisol did not respond, while the other steroids responded in the order of 17-OH P>E(2)> testosterone. We conclude that there were three different groups of TAR when the patient was already 10 years old: (i) ACTH-sensitive (the majority), (ii) partially ACTH-insensitive but LH/hCG-sensitive (three nodules in the left testis and three in the right testis), (iii) almost entirely ACTH-insensitive and partially hCG-insensitive (a single nodule in the right testis). Probably, the never suppressed gonadotropin levels (presumably due to the bilateral testicular damage subsequent to the cryptorchid state) and the hCG therapy were major etiological factors for the appearance of the second and third population of TAR.
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2/60. Persistent Mullerian duct syndrome in adult: a case report.

    A twenty-eight year old phenotypically and genotypically normal male adult was admitted with a right inguinal hernia, a right retractile testis and left cryptorchidism. During surgery, he was found to have a uterus in the right spermatic cord and a left undescended testis (intra abdominal type). The uterus was excised, left orchidectomy, right orchidoplexy and right herniorrhaphy were performed. This rare case is reported as persistent Mullerian Duct syndrome (PMDS) which is caused by impaired action of Mullerian Inhibiting Substance (MIS) in regressing the Mullerian duct.
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3/60. Persistent mullerian duct syndrome with teratoma in an ectopic testis: imaging features.

    The persistent mullerian duct syndrome represents a rare form of male pseudohermaphroditism, secondary to mullerian inhibiting factor (MIF) deficiency. We describe imaging findings in a 30-year-old male (46 XY karyotype) with bilateral cryptorchidism and mullerian duct anomalies (presence of uterus and fallopian tubes). Grade-III teratoma with yolk sac tumour was detected in one of the undescended testis, lying in the pelvic cavity. The other testis was in the inguinal canal. The rest of the wolffian duct structures (e.g. prostate, seminal vesicles) were nearly normal. Very few reports of imaging findings of this entity have been published thus far, probably because of the rarity of entity, incidental detection of most of the cases at surgery and relatively asymptomatic clinical presentation.
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4/60. Pitfalls of conventional human chorionic gonadotropin stimulation test to detect hormonally functional cryptorchid testes in midchildhood.

    OBJECTIVE: To report two cases misdiagnosed as bilateral anorchism in midchildhood on the basis of multiple conventional human chorionic gonadotropin (HCG) stimulation tests and sonograms of the abdomen and pelvis. methods: In two young male patients with cryptorchidism who were considered to have anorchism, we describe the findings on clinical examination, the testosterone levels before and after standard HCG stimulation testing, and sonographic findings during the midchildhood period. In both cases, as the children approached puberty the diagnosis was found to be incorrect. RESULTS: Two boys, 8 and 91/2 years old, were seen in consultation in our Pediatric Endocrine Clinic with a presumed diagnosis of anorchism. In the first case, multiple conventional HCG stimulation tests were done. In the second case, a single stimulation test was performed during routine follow-up assessments. In both cases, testosterone levels before and after HCG stimulation were consistent with the diagnosis of absent functional testicular tissue. Sonograms of the abdomen and pelvis also failed to detect the testicles. Both patients were ultimately noted to be pubertal (at 14 1/2 and >13 1/2 years, respectively) and to have early pubertal testosterone levels. A testicle was detected in one patient by abdominal computed tomographic scan and in the other by palpation of the inguinal canal. CONCLUSION: Conventional dosing and duration of the HCG stimulation test, as widely recommended in standard textbooks and in articles in the medical literature, may not elicit positive HCG-induced testosterone responses during midchildhood for detection of functional testicular tissue. During the midchildhood period, which is characterized by low gonadotropin, low sex steroid production, and a highly sensitive hypothalamic-pituitary-gonadal axis to feedback inhibition, a prolonged HCG stimulation test-perhaps of 4 to 6 weeks' duration-may be necessary. In addition, other investigational modalities may need to be used to detect the presence of functional testicular tissue during this developmental period.
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5/60. A case of bilateral seminoma in the setting of persistent mullerian duct syndrome.

    We are reporting on the case of a 46XY male patient that presented a bilateral testicular seminoma in the setting of bilateral cryptorchidia and left inguinal hernia. The patient received five courses of cisplatin/ciclophosphamide followed by 3,000 cGy of radiation. The patient then, underwent resection of the residual mass and of an unrecognizable pelvic structure. The histopathological analysis revealed necrosis of the residual seminoma and an atrophic uterus. Currently, the patient is alive and asymptomatic at 88 months of follow-up. This is the third case reported of a patient with persistent mullerian duct syndrome and bilateral seminoma.
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6/60. Complicated airway management in a child with prune-belly syndrome.

    We describe a 15-month-old boy with prune-belly syndrome (PBS) in whom airway management was complicated. Following an inhalation induction using sevoflurane, tracheal intubation by direct laryngoscopy proved impossible after repeated attempts. A laryngeal mask airway (LMAtrade mark) was inserted and the child had an uneventful anaesthetic course.
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7/60. Persistent Mullerian duct syndrome presenting with bilateral intra-abdominal gonadal tumours and obstructive uropathy.

    Persistent Mullerian duct syndrome is a rare, autosomal recessive intersex disorder characterized by the presence of completely developed Mullerian duct derivatives in an otherwise normally virilized male with a 46, XY karyotype. We discuss a rare presentation of this disorder, bilateral gonadal tumours with obstructive uropathy, and its management, together with a review of the literature.
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8/60. Bidirectional Glenn shunt in an infant with prune-belly syndrome.

    The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.
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9/60. A novel mutation in the anti-mullerian hormone gene as cause of persistent mullerian duct syndrome.

    Persistent mullerian duct syndrome is a relatively rare inherited defect of sexual differentiation characterised by failure of regression of the mullerian ducts in males. In affected individuals, uterus and tubes are present because of defects of synthesis or action of anti-mullerian hormone (AMH), normally produced by the sertoli cells of the testis. patients are normally virilised, although mono- or bilateral cryptorchidism may be present. We observed two brothers (chromosomes 46 XY), aged 11 years and 2 months and 8 years and 3 months respectively, with bilateral cryptorchidism. The diagnosis of persistent mullerian duct syndrome was made on the basis of laparoscopic evidence of uterus and tubes, undetectable plasma levels of AMH and a 23 base pair duplicative insertion in exon 5 of the AMH gene, causing the introduction of a premature stop codon, homozygous in the two brothers. The surgical correction of the genital abnormalities was successfully carried out by laparoscopic orchidopexy according to Fowler-Stephens. CONCLUSION: Persistent mullerian duct syndrome should be taken into consideration in all cases of bilateral cryptorchidism. laparoscopy is the elective procedure for diagnosis of this disease and laparoscopic surgery for orchidopexy of intra-abdominal testes. mutation analysis of the anti-mullerian hormone gene in these patients helps to understand the structure-function relationship of the anti-mullerian hormone protein, although it is not clear at present whether anti-mullerian hormone is necessary to maintain normal testicular function.
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10/60. Persistent mullerian duct syndrome and prostate cancer.

    A localized Gleason score 6 (3 3) prostate cancer was found in a 56-year-old man with bilateral cryptorchidism. Radical laparoscopic prostatectomy was performed. However, at the beginning of the procedure, a uterus, two fallopian tubes, and two intra-abdominal gonads were endoscopically identified. The mullerian rests were excised and the gonads biopsied. The histologic examination showed testicular tissue. The association of prostate cancer and persistent mullerian duct syndrome to our knowledge has not previously been reported. Prostate cancer is an androgen-dependent neoplasm, and patients with male pseudohermaphroditism have poor androgen production that should provide protection against it.
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