1/6. Progestin binding in testes from three siblings with the syndrome of male pseudohermaphroditism with testicular feminization.We have found a specific binding protein for synthetic progestins 6,7-[3H]methyltrienolone (R1881) and 17,21-dimethyl-19-norpregna-4,9-diene-3,20-dione (R5020) and in the testis cytosol from three "sisters" with the complete form of the testicular feminization syndrome. The binding component sediments in the 8S region of sucrose gradients. It is saturable. The apparent affinity constant (Ka) for R5020 was determined in two cases and found to be 1.8 and 0.6 X 10(8) M-1. The number of binding sites calculated from Scatchard plots is relatively high: 572 and 826 fmol/mg protein. Competition studies indicate that this putative receptor is specific for natural and synthetic progestins but not for 5 alpha-dihydrotestosterone and cortisol. Similar progestin binding could not be found in normal human and rat testes.- - - - - - - - - - ranking = 1keywords = testis (Clic here for more details about this article) |
2/6. Endocrine investigations in two cases of feminizing Leydig cell tumour.Two patients, aged 32 and 35 years, presented with gynaecomastia and a unilateral testicular tumour which proved to be a Leydig cell tumour. Pre-operative samples taken at 08.00 h on different days showed marked elevation of plasma oestradiol in the first patient, and very slight irregular oestradiol elevation in the second, plasma oestrone within the normal range in both patients, reduced plasma testosterone in the first patient and reduced or normal testosterone in the second, and low or low-normal serum LH and FSH in both patients. One of the patients received an oral dose of 100 mg of clomiphene citrate for 3 consecutive days which induced a rise in LH and FSH and a decrease in the 17-hydroxyprogesterone/androstenedione ratio. These data suggest the inhibiting effect of endogenous hyperoestrogenism on testicular steroidogenesis owing to both the reduction of gonadotropin secretion and a direct local negative effect on C 17,20-lyase. After human chorionic gonadotropin stimulation, oestradiol response was increased and abnormally prolonged, a finding which may be helpful when diagnosing a feminizing Leydig cell tumour; testosterone reached normal values. After removal of the tumoural testis, gynaecomastia regressed within a few days, gonadotropins increased, oestrogens dropped, testosterone and 5 alpha-dihydrotestosterone normalized in one patient but remained low in the other at day 30. The leydig cells outside the tumour appeared morphologically normal, but the count gave evidence of juxtatumoural Leydig cell hyperplasia in areas where the tumour was well encapsulated while showing a significant reduction at a distance from the tumour and in the contralateral testis by comparison with control testes.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 2keywords = testis (Clic here for more details about this article) |
3/6. An unbalanced autosomal translocation (7;9) associated with feminization.A newborn girl presented with generalized mild dysmorphic features. She later developed heart failure and hydrocephalus, and died aged 5 months. Chromosome analysis revealed an unbalanced reciprocal translocation (with partial trisomy for half of the long arm of 7 and partial monosomy for the short arm of chromosome 9) and normal but inappropriate sex chromosomes (XY). The karyotype (46,XY,-9, der(9),t(7;9)(q31.1;p23)pat) was inferred from her father who was a balanced carrier: 46, XY,(7;9)(q31.1;p23). The evidence of the present case, when considered with that of previous reports, suggests that deletion of genes on the 9p may have caused the feminization and therefore that the 9p region may contain genes which are important in the normal process of testis formation.- - - - - - - - - - ranking = 1keywords = testis (Clic here for more details about this article) |
4/6. Feminizing sertoli cell tumor associated with peutz-jeghers syndrome (histologic and ultrastructural study).A case of peutz-jeghers syndrome associated with sertoli cell tumor of the testis and bilateral gynecomasty in an 8-year-old boy is described. The authors emphasize the unusual histologic and ultrastructural features of the tumor and discuss the physiopathologic aspects of the present case.- - - - - - - - - - ranking = 1keywords = testis (Clic here for more details about this article) |
5/6. Feminizing Sertoli cell tumors in boys with peutz-jeghers syndrome.We report the pathology findings in two cases of multicentric Sertoli cell testicular tumors in two young boys with probable peutz-jeghers syndrome. Four cases of such tumors occurring in boys with peutz-jeghers syndrome were previously reported. Each of the two boys reported in this paper had prominent gynecomastia, rapid growth, and advanced bone age. serum levels of estradiol were markedly elevated. anti-mullerian hormone was measured in the serum of one of the boys and was in the normal range for age. Bilateral orchiectomy was performed in each case because the neoplastic growth would most likely result in sterility, and curtailment of height potential was threatened from continued elevation of estradiol levels. Microscopically, greatly enlarged seminiferous tubules packed with ovoid Sertoli-like cells were present. Prominent eosinophilic basement membrane surrounded the tubules and intersected between the cells, forming hyalinized ovoid globules and microcalcifications. Ultrastructure revealed lamination of basement membranes surrounding adjacent cells, ovoid cells with abundant cytoplasm, and limited smooth endoplasmic reticulum. Studies of testicular tumor tissue from both cases revealed increased transcription of the aromatase cytochrome P450 gene using promoter II, the promoter directing aromatase expression in the normal ovary and testis. The levels of transcripts were comparable to corpus luteum, thus resulting in increased estrogen synthesis. Transcripts specific for placental-type aromatase promoters (I.1 and I.2) were not detected in significant levels in these tumors.- - - - - - - - - - ranking = 1keywords = testis (Clic here for more details about this article) |
6/6. Bilateral sertoli cell tumor of testes with feminizing and masculinizing activity occurring in a child.A case of an unusual, histologically atypical, bilateral sertoli cell tumor of testis, in association with masculinization and feminization effects, that occurring in a 5-year-old boy, is presented. Although Sertoli cell tumors are not infrequent, this case is morphologically and endocrinologically unique.- - - - - - - - - - ranking = 1keywords = testis (Clic here for more details about this article) |