1/5. Testicular feminization syndrome in the neonate.If we see a young, phenotypically female patient with an XY karyotype, it is of great importance to differentiate between the testicular feminization syndrome and gonadal dysgenesis. patients with testicular feminization will always have normal testes, which are situated either in the ovarian fossa or in the inguinal canal. patients with gonadal dysgenesis always have streak gonads. The risk of developing a malignancy in an abnormally located testis is very low, certainly before puberty, whereas the risk for dysgenetic gonads to develop a malignancy is high. Testes in patients with testicular feminization have an important endocrine function in puberty, whereas in gonadal dysgenesis patients they do not. For these reasons, in patients with testicular feminization, one should not remove the testes until the completion of puberty, whereas in patients with gonadal dysgenesis removal should be performed immediately upon recognition of the disorder.- - - - - - - - - - ranking = 1keywords = gonadal dysgenesis, dysgenesis (Clic here for more details about this article) |
2/5. Dysgerminomas (seminomas) in genetic males with female phenotype. One case of gonadal dysgenesis and gonadoblastoma and one of testicular feminization.Gonadal tumors in patients with somatosexual ambiguity are rare but of considerable clinical and pathological interest. Two patients with dysgerminomas and abnormal sexual differentiation were recently treated at the Department of Gynecologic Oncology. One (Case 1) was an 18-year old girl and the other (Case 2) an unmarried woman of 58 years. In spite of their female phenotype both showed a male genotype, 46, XY. In Case 1 the dysgerminoma (or rather seminoma) originated in a gonadoblastoma in one of two dysgenetic testes but in Case 2 in one of two "feminizing testes". The clinico-pathological features of both cases will be reported and commented and the relation between the germ cell tumors, gonadal dysgenesis and the Y-chromosome will be discussed.- - - - - - - - - - ranking = 1.25keywords = gonadal dysgenesis, dysgenesis (Clic here for more details about this article) |
3/5. Gonadotropin regulation during puberty in complete androgen insensitivity syndrome with testicles in situ.This paper describes the time course of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels in two patients with the complete androgen insensitivity syndrome (AIS) during puberty and adolescence prior to gonadectomy. In early puberty, LH values were comparable to normal male as well as female standards. At the age of 17 and 18.7 years, respectively, a sudden rise in LH occurred just after the start of estradiol supplementation. These high LH concentrations in the late puberty of our patients are comparable to the values found during early puberty in castrated individuals and children with gonadal dysgenesis. In contrast, FSH concentrations continuously showed levels in the normal male or female range with no rise during adolescence. The sudden increase of LH in late puberty is most probably due to defective testosterone receptors in the pituitary/hypothalamus which apparently can no longer be suppressed by rising testosterone levels and an escape from the negative estradiol feedback. The role of estradiol supplementation might be incidental and further investigation into its role is needed. In contrast to the elevated levels of FSH in patients with defective spermatogenesis, FSH is normal in our patients with AIS and testes in situ. This indicates that FSH secretion is regulated by a combined action of estradiol and gonadal hormones like inhibin.- - - - - - - - - - ranking = 0.25keywords = gonadal dysgenesis, dysgenesis (Clic here for more details about this article) |
4/5. Digital blunt dissection technique to assist laparoscopic gonadectomy in inguinally located/adhered gonads.Laparoscopic gonadal removal in two patients, one with XY gonadal dysgenesis and the other with complete androgen insensitivity is described. In both cases, the gonads were either located deep in the inguinal canal or adherent in that area. Digital blunt dissection using a finger passed through the lateral lower abdominal trocar incision site (12 mm) at the mid-clavicular line of the lower abdomen, combined with external palpation/elevation of the inguinal ring, allowed rapid dissection and isolation of the desired structures and vascular pedicles.- - - - - - - - - - ranking = 0.25keywords = gonadal dysgenesis, dysgenesis (Clic here for more details about this article) |
5/5. Removal of gonads in Y-chromosome-bearing gonadal dysgenesis and in androgen insensitivity syndrome by laparoscopic surgery.BACKGROUND. This paper addresses the value of laparoscopic surgery for the removal of gonads in patients with Y-chromosome-bearing gonadal dysgenesis and androgen insensitivity syndrome, who are otherwise faced with a high rate of gonadal malignancy. methods. Three patients with Y-chromosome-bearing gonadal dysgenesis and one patient with androgen insensitivity syndrome were operated upon laparoscopically. Removal of gonads was accomplished by their mobilization and dissection from the pelvic side walls, with ligation and transection of the utero-ovarian and infundibulopelvic ligaments. RESULTS. Surgery was without complications. Histological examination of the gonads showed complete removal and absence of malignancy in each patient. patients were discharged the day after surgery. CONCLUSIONS. The laparoscopic approach is a safe and effective alternative to laparotomy in the management of patients with Y-chromosome-bearing gonadal dysgenesis and with androgen insensitivity syndrome.- - - - - - - - - - ranking = 1.75keywords = gonadal dysgenesis, dysgenesis (Clic here for more details about this article) |