BACKGROUND Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of theovary. It was first reported and named in 1973. These tumors typically presentwith pelvic/abdominal pain and tenderness, a ma...BACKGROUND Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of theovary. It was first reported and named in 1973. These tumors typically presentwith pelvic/abdominal pain and tenderness, a mass, and/or abnormal menses,but rarely present with masculinity in children and adolescents. Only 2 cases ofthese tumors have been reported in premenarchal girls, who demonstratedhormonal activity, with a history of the development of a virilizing female due tohyperandrogenism. Here, we report a case of a giant SST with obviousmasculinity combined with Meig’s syndrome and CA125 elevation.CASE SUMMARY A 17-year-old female presented with a 7-year history of the development ofmasculinity and a 2-year history of amenorrhea. She had hirsutism, acne, obviouslaryngeal prominence, and voice deepening. Physical examination showed a malesuprapubic hair pattern and a 4.0 cm × 1.5 cm enlarged clitoris. Laboratory testsshowed that the testosterone level was > 15.00 ng/mL (normal range: 0.14-0.76ng/mL), and androstenedione level was > 10.00 ng/mL (normal range: 0.3-3.3ng/mL). A computed tomography scan of the abdomen and pelvis was carriedout and showed a large, solid and cystic, partly calcified pelvic mass in the rightovary measuring 27.1 cm × 20.0 cm × 11.0 cm, 15 cm above the umbilicus (to thelevel of the upper part of L1). Intraoperative findings at laparotomy revealed alarge tumor arising from the right ovary. Approximately, 500 mL of pale-yellow clear liquid was found in the pelvic cavity. A right salpingo-oophorectomy wasperformed. Microscopic examination and immunohistochemical staining of thesurgical specimen showed an SST of the ovary.CONCLUSION This report is remarkable as our patient was not only diagnosed with an SST ofthe ovary, which is extremely rare in this age group, but was the largest and mostobvious reported patient with this tumor who presented with virilization.Therefore, gynecologists should be aware of this potential complication inadolescent girls with a mass in the ovary.展开更多
Sertoli-Leydig cell tumors (SLCTs) are rare tumors. Mass and pain are the presenting feature. The varying histopathology and differentiation of this tumor present difficulties with proper diagnosis and development of ...Sertoli-Leydig cell tumors (SLCTs) are rare tumors. Mass and pain are the presenting feature. The varying histopathology and differentiation of this tumor present difficulties with proper diagnosis and development of optimal treatment regimens. The prognosis depends on tumors grading and staging. Surgery is main stay management option. Chemotherapy and radiation options are still of choice.?We aimed?to?present Sertoli-Leydig cell tumor managed surgical at Latifa Hospital in Dubai, UAE with acceptable outcome and good patient satisfaction.展开更多
Objective: To inquire into diagnosis, and treatment of virilizing andfeminizing a-drenal syndrome, differential diagnosis between benign and malignant sex hormoneproducing adrenal neo-plasma and, treatment principles ...Objective: To inquire into diagnosis, and treatment of virilizing andfeminizing a-drenal syndrome, differential diagnosis between benign and malignant sex hormoneproducing adrenal neo-plasma and, treatment principles of congenital adrenal hyperplasia (CAH).Methods: Eight cases of CAH and 5 cases of sex hormone producing adrenal neoplasma were admitted tohospital during 1986-1996. The former included 3 rare cases of 17 a hydroxylase deficiency. Thelatter included 3 cases of feminizing adrenal tumor and 2 cases of virilizing adrenal tumor.Results: Weight, size and CT of the tumor, DHEA, 17 -ks, sex hormone levels, infiltration, andmetastasis were closely related to the degree of differentiation of the tumors. Conclusion:Virilizing and feminizing adrenal neoplasm were removed surgically by different incisions. Modifiedsubcostal incision was recommended as the best choice for huge adrenal mass. Corticoadrenal hormonetreatment fa CAH should be individualized according to the different types of the disease. Sexhormones were not suitable for children suffering from 17 hydroxylase deficiency before puberty.展开更多
BACKGROUND Turner syndrome(TS)has a variety of different karyotypes,with a wide range of phenotypic features,but the specific karyotype may not always predict the phenotype.TS with Y chromosome mosaicism may have mixe...BACKGROUND Turner syndrome(TS)has a variety of different karyotypes,with a wide range of phenotypic features,but the specific karyotype may not always predict the phenotype.TS with Y chromosome mosaicism may have mixed gonadal dysgenesis,and the mosaicism is related to the potential for gonadoblastoma.CASE SUMMARY In this case report,we report two cases of TS with different karyotypes and gonadal dysgenesis.Patient 1 had obvious virilization,and was positive for the SRY gene,but her karyotype in peripheral blood lymphocytes was 45X.Patient 2 had a mosaic karyotype,45X/46X,dic(Y:Y)(p11.3:p11.2),and the proportion of Y-bearing cells was 50%in peripheral blood lymphocytes,but the patient had normal female external genitalia and streaky gonads,with no genital virilism.Different tissues in the same TS individual may exhibit different ratios of mosaicism.The gonadal determination and differentiation of mosaic TS are primarily dependent on the predominant cell line in the gonads.CONCLUSION In TS patients with virilization,it is necessary to test at least two to three tissues to search for cryptic Y material.展开更多
Objective:To study the clinical characteristics of testosterone-producing ovarian tumors.Methods:Twenty-five patients with testosterone-producing ovarian tumors diagnosed pathologically were treated in Peking Union Me...Objective:To study the clinical characteristics of testosterone-producing ovarian tumors.Methods:Twenty-five patients with testosterone-producing ovarian tumors diagnosed pathologically were treated in Peking Union Medical College Hospital from Jan 1986 to Oct 2006.Clinical documents of the 25cases were analyzed retrospectively.Results:(1)The median age of patients was 27 years.Menstrual irregularity was seen in 96% of patients and virilization was seen in 100%.(2)Endocrinological tests:Total testosterone,LH,FSH,LH/FSH,E2 in serum before and after operation were 16.9±6.8/1.0±1.6 nmol/L(P=0.000),6.6±3.41/11.7±6.8 IU/L(P=0.025),4.9±2.6/9.2±7.8 IU/L(P=0.072),2.1±2.5/1.7±1.0(P=0.579),241±139/164±161 pmol/L(P=0.131),respectively.Initial investigation showed the level of E2 was in early follicle stage in 86% of patients,normal level of ACTH in 92%,increased level of17-αOHP and F in 50% and in 22% of patients,respectively.all patients have normal urinary free cortisol collected over a 24-hour period.Dexamethasone suppression tests and ACTH stimulating tests suggested non-adrenocorticotrophic homone dependency of the hyperandrogenic state.(3)Pathological features:of all tumors,60% were Sertoli-Leydig cell tumors in which 67% of them were poor differentiated,32%were lipid cell tumors in which all of them were well differentiated.All tumors were unilateral.The median size of tumors was 4.8cm.On cut surface most of them were solid and yellow.(4)Treatment and prognosis:twenty-two younger patients were performed conservative staging surgery.Patients with poor-differentiated tumors accepted chemotherapy and GnRH-a.After removal of ovarian tumors,symptoms of virilization were improved and five patients had healthy children.Conclusions:Testosterone-producing ovarian tumors are often seen in reproductive ages.They produce defeminization followed by virilization.The total level of testosterone in serum was increased markedly while decreased rapidly after removal of tumor.Owing to their relatively small size,the detectio展开更多
Gon adotropin therapy is comm only used to in duce virilizati on and spermatoge nesis in male isolated hypog on adotropic hypog on adism (IHH) patients. In clinical practice, 5.6%-15.0% of male IHH patients show poor ...Gon adotropin therapy is comm only used to in duce virilizati on and spermatoge nesis in male isolated hypog on adotropic hypog on adism (IHH) patients. In clinical practice, 5.6%-15.0% of male IHH patients show poor responses to gonadotropin treatment;therefore, testosterone (T) suppleme ntation can serve as an alter native therapy to no rmalize serum T levels and promote virilization. However, treatment with exogenous T impairs spermatogenesis and suppresses intratesticular T levels. This retrospective study aimed to determine whether oral testosterone undeca noate (TU) suppleme ntation together with human chorionic gonadotropin (hCG) would negatively affect spermatogenesis in IHH patients compared with hCG alone. One hundred and seven IHH patients were included in our study. Fifty-four patients received intramuscular hCG and oral TU, and 53 patients received intramuscular hCG alone. The median follow-up time was 29 (range: 12-72) mon ths in both groups. Compared with the hCG group, the hCG/TU group required a shorter median time to normalize serum T levels (P < 0.001) and achieve Tanner stage (III and V) of pubic hair and genital development (P < 0.05). However, there were no significant differences in the rate of seminal spermatozoa appearance, sperm concentration, or median time to achieve different sperm concentration thresholds between the groups. In addition, there were no significant differences in side effects, such as acne and gynecomastia, observed in both groups. This study indicates that oral TU supplementation together with hCG does not impair spermatogenesis in treated IHH patients compared with hCG alone, and it shortens the time to normalize serum T levels and promote virilization.展开更多
文摘BACKGROUND Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of theovary. It was first reported and named in 1973. These tumors typically presentwith pelvic/abdominal pain and tenderness, a mass, and/or abnormal menses,but rarely present with masculinity in children and adolescents. Only 2 cases ofthese tumors have been reported in premenarchal girls, who demonstratedhormonal activity, with a history of the development of a virilizing female due tohyperandrogenism. Here, we report a case of a giant SST with obviousmasculinity combined with Meig’s syndrome and CA125 elevation.CASE SUMMARY A 17-year-old female presented with a 7-year history of the development ofmasculinity and a 2-year history of amenorrhea. She had hirsutism, acne, obviouslaryngeal prominence, and voice deepening. Physical examination showed a malesuprapubic hair pattern and a 4.0 cm × 1.5 cm enlarged clitoris. Laboratory testsshowed that the testosterone level was > 15.00 ng/mL (normal range: 0.14-0.76ng/mL), and androstenedione level was > 10.00 ng/mL (normal range: 0.3-3.3ng/mL). A computed tomography scan of the abdomen and pelvis was carriedout and showed a large, solid and cystic, partly calcified pelvic mass in the rightovary measuring 27.1 cm × 20.0 cm × 11.0 cm, 15 cm above the umbilicus (to thelevel of the upper part of L1). Intraoperative findings at laparotomy revealed alarge tumor arising from the right ovary. Approximately, 500 mL of pale-yellow clear liquid was found in the pelvic cavity. A right salpingo-oophorectomy wasperformed. Microscopic examination and immunohistochemical staining of thesurgical specimen showed an SST of the ovary.CONCLUSION This report is remarkable as our patient was not only diagnosed with an SST ofthe ovary, which is extremely rare in this age group, but was the largest and mostobvious reported patient with this tumor who presented with virilization.Therefore, gynecologists should be aware of this potential complication inadolescent girls with a mass in the ovary.
文摘Sertoli-Leydig cell tumors (SLCTs) are rare tumors. Mass and pain are the presenting feature. The varying histopathology and differentiation of this tumor present difficulties with proper diagnosis and development of optimal treatment regimens. The prognosis depends on tumors grading and staging. Surgery is main stay management option. Chemotherapy and radiation options are still of choice.?We aimed?to?present Sertoli-Leydig cell tumor managed surgical at Latifa Hospital in Dubai, UAE with acceptable outcome and good patient satisfaction.
文摘Objective: To inquire into diagnosis, and treatment of virilizing andfeminizing a-drenal syndrome, differential diagnosis between benign and malignant sex hormoneproducing adrenal neo-plasma and, treatment principles of congenital adrenal hyperplasia (CAH).Methods: Eight cases of CAH and 5 cases of sex hormone producing adrenal neoplasma were admitted tohospital during 1986-1996. The former included 3 rare cases of 17 a hydroxylase deficiency. Thelatter included 3 cases of feminizing adrenal tumor and 2 cases of virilizing adrenal tumor.Results: Weight, size and CT of the tumor, DHEA, 17 -ks, sex hormone levels, infiltration, andmetastasis were closely related to the degree of differentiation of the tumors. Conclusion:Virilizing and feminizing adrenal neoplasm were removed surgically by different incisions. Modifiedsubcostal incision was recommended as the best choice for huge adrenal mass. Corticoadrenal hormonetreatment fa CAH should be individualized according to the different types of the disease. Sexhormones were not suitable for children suffering from 17 hydroxylase deficiency before puberty.
文摘BACKGROUND Turner syndrome(TS)has a variety of different karyotypes,with a wide range of phenotypic features,but the specific karyotype may not always predict the phenotype.TS with Y chromosome mosaicism may have mixed gonadal dysgenesis,and the mosaicism is related to the potential for gonadoblastoma.CASE SUMMARY In this case report,we report two cases of TS with different karyotypes and gonadal dysgenesis.Patient 1 had obvious virilization,and was positive for the SRY gene,but her karyotype in peripheral blood lymphocytes was 45X.Patient 2 had a mosaic karyotype,45X/46X,dic(Y:Y)(p11.3:p11.2),and the proportion of Y-bearing cells was 50%in peripheral blood lymphocytes,but the patient had normal female external genitalia and streaky gonads,with no genital virilism.Different tissues in the same TS individual may exhibit different ratios of mosaicism.The gonadal determination and differentiation of mosaic TS are primarily dependent on the predominant cell line in the gonads.CONCLUSION In TS patients with virilization,it is necessary to test at least two to three tissues to search for cryptic Y material.
文摘Objective:To study the clinical characteristics of testosterone-producing ovarian tumors.Methods:Twenty-five patients with testosterone-producing ovarian tumors diagnosed pathologically were treated in Peking Union Medical College Hospital from Jan 1986 to Oct 2006.Clinical documents of the 25cases were analyzed retrospectively.Results:(1)The median age of patients was 27 years.Menstrual irregularity was seen in 96% of patients and virilization was seen in 100%.(2)Endocrinological tests:Total testosterone,LH,FSH,LH/FSH,E2 in serum before and after operation were 16.9±6.8/1.0±1.6 nmol/L(P=0.000),6.6±3.41/11.7±6.8 IU/L(P=0.025),4.9±2.6/9.2±7.8 IU/L(P=0.072),2.1±2.5/1.7±1.0(P=0.579),241±139/164±161 pmol/L(P=0.131),respectively.Initial investigation showed the level of E2 was in early follicle stage in 86% of patients,normal level of ACTH in 92%,increased level of17-αOHP and F in 50% and in 22% of patients,respectively.all patients have normal urinary free cortisol collected over a 24-hour period.Dexamethasone suppression tests and ACTH stimulating tests suggested non-adrenocorticotrophic homone dependency of the hyperandrogenic state.(3)Pathological features:of all tumors,60% were Sertoli-Leydig cell tumors in which 67% of them were poor differentiated,32%were lipid cell tumors in which all of them were well differentiated.All tumors were unilateral.The median size of tumors was 4.8cm.On cut surface most of them were solid and yellow.(4)Treatment and prognosis:twenty-two younger patients were performed conservative staging surgery.Patients with poor-differentiated tumors accepted chemotherapy and GnRH-a.After removal of ovarian tumors,symptoms of virilization were improved and five patients had healthy children.Conclusions:Testosterone-producing ovarian tumors are often seen in reproductive ages.They produce defeminization followed by virilization.The total level of testosterone in serum was increased markedly while decreased rapidly after removal of tumor.Owing to their relatively small size,the detectio
基金the grant from the National Natural Science Foundation of China (Project No. 81671443, 81601270).
文摘Gon adotropin therapy is comm only used to in duce virilizati on and spermatoge nesis in male isolated hypog on adotropic hypog on adism (IHH) patients. In clinical practice, 5.6%-15.0% of male IHH patients show poor responses to gonadotropin treatment;therefore, testosterone (T) suppleme ntation can serve as an alter native therapy to no rmalize serum T levels and promote virilization. However, treatment with exogenous T impairs spermatogenesis and suppresses intratesticular T levels. This retrospective study aimed to determine whether oral testosterone undeca noate (TU) suppleme ntation together with human chorionic gonadotropin (hCG) would negatively affect spermatogenesis in IHH patients compared with hCG alone. One hundred and seven IHH patients were included in our study. Fifty-four patients received intramuscular hCG and oral TU, and 53 patients received intramuscular hCG alone. The median follow-up time was 29 (range: 12-72) mon ths in both groups. Compared with the hCG group, the hCG/TU group required a shorter median time to normalize serum T levels (P < 0.001) and achieve Tanner stage (III and V) of pubic hair and genital development (P < 0.05). However, there were no significant differences in the rate of seminal spermatozoa appearance, sperm concentration, or median time to achieve different sperm concentration thresholds between the groups. In addition, there were no significant differences in side effects, such as acne and gynecomastia, observed in both groups. This study indicates that oral TU supplementation together with hCG does not impair spermatogenesis in treated IHH patients compared with hCG alone, and it shortens the time to normalize serum T levels and promote virilization.
