Severe hyperandrogenemia in postmenopausal woman as a presentation of ovarian hyperthecosis. Case report and mini review of the literature

Ovarian hyperthecosis (OH) is characterized by the presence of abundant luteinized theca cells in ovaries that secret androgen. It typically presents as severe hyperandrogenism and/or virilization in postmenopausal woman. Here we describe a 66-year old woman with presentation of severe hirsutism, alopecia, clitoromegaly and laboratory finding of significantly elevated serum total testosterone concentration and hyperinsulinemia. Performed imaging studies revealed normal sized, homogeneous ovaries, signs of endometrial hypertrophy and normal adrenal glands. Due to severe hyperandrogenemia and signs of endometrial hypertrophy, the total abdominal hysterectomy with bilateral salpingo-oophorectomy has been performed. Pathological examination revealed OH and endometrial hyperplasia. Androgenic activity of ovarian stromal cells has been confirmed using alpha-inhibin histochemical staining. Postmenopausal hyperandrogenemia is a diagnostic and therapeutic challenge and the imaging studies often may be misleading and require careful and critical consideration.     

Virilization due to ovarian hyperthecosis in a postmenopausal woman

A 51-year-old woman presented with hirsutism and virilization of gradual onset. The serum gonadotropin concentrations were in the postmenopausal range, the serum testosterone concentration was markedly elevated (9.8 nmol/l) and the serum estradiol concentration (220 pmol/l) was elevated above the postmenopausal range. A selective venous catheterization study demonstrated raised serum testosterone and androstenedione levels in ovarian veins and suggested the presence of a left ovarian tumor. The raised peripheral estradiol level was shown to be due to ovarian hypersecretion. After bilateral oophorectomy the serum testosterone became normal. Ovarian histology revealed bilateral stromal hyperthecosis. Ovarian hyperthecosis is a rare but important cause of serum testosterone levels in the neoplastic range. This is the third case reported of postmenopausal virilization due to ovarian hyperthecosis and the first report of a selective venous catheterization study in such a patient.     

Psychosexual aspects of virilization syndrome

A case is reported of a 35-year-old black woman with marked hirsuitism. The focus is on the psychosexual aspects of her illness. Physicians must attend to the potentially devastating effects of virilization syndrome on their female patients.     

An ovarian steroid cell tumor with virilization and hypothyroidism: a case report

Steroid cell tumors are rare ovarian sex-cord stromal tumors with malignant potential. The majority of these tumors produce steroids with testosterone being the most common. The diagnosis of these rare tumors can be problematic especially in the case of a small nonpalpable ovarian tumor. A 41-year-old woman presented with the gradual onset of defeminization, mild hypothyroidism, and virilization. A physical examination revealed a relatively healthy woman with borderline hirsutism, clitoromegaly and adnexal fullness without a palpable tumor. Elevated serum levels of TSH and testosterone were found preoperatively. Five weeks after an oophorectomy, serum levels of TSH and testosterone returned to normal and menstruation returned. Nonpalpable virilizing ovarian steroid cell tumors may be difficult to diagnose. A careful study of the patient’s history, the development of defeminization followed by virilization, and a “characteristic” ultrasonogram, can be helpful for diagnosis. Hormonal studies including thyroid function should also be considered in an ovarian steroid cell tumor.     

具有分泌性激素功能的卵巢肿瘤50例分析

目的探讨具有分泌性激素功能的卵巢肿瘤的临床特征与内分泌症状,为正确诊断提供临床参考资料。方法回顾性分析1980年1月至2003年1月在北京协和医院就诊的具有分泌性激素功能的卵巢肿瘤50例,分别对症状、肿瘤种类、性激素等相互间关系进行统计学分析。结果50例中以卵巢颗粒细胞瘤为多见,其次为卵巢支持间质细胞瘤等。主要为雄激素与雌激素增多症状,其中雄激素异常的常见临床表现依次为多毛、阴蒂增大与喉结出现。多毛为首发表现,多见于卵巢支持间质细胞瘤与卵巢类脂质细胞瘤。雌激素异常的主要临床表现为绝经后出血与月经紊乱,多见于颗粒细胞瘤。结论多毛、阴蒂增大等是雄激素过多的最常见表现,睾酮≥6.0nmol/L者应高度怀疑发生肿瘤的可能,治疗以手术为主。     

Maternal virilization due to mucinous ovarian tumour: a case report

Virilization in pregnancy is rare and mostly due to luteoma or to hyper-reactio luteinalis. We present a rare case of a virilization borderline mucinous ovarian tumour on a gravida 1 patient. The tumour was responsible for a clinical hyperandrogenism and for an increased level of testosterone. This patient was treated by ovariectomy at 31 weeks of gestation. The surgery was completed one month after delivery. There was no fetal consequence and the clinical and biological signs of virilization totally disappeared after surgery.     

A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors

Objective

To evaluate the clinicopathological features of ovarian Sertoli-Leydig cell tumors (SLCTs) and to explore the reasonable therapy.

Methods

A total of 40 cases of SLCTs were retrospectively reviewed.

Results

The incidence of SLCTs was 0.41%, with a median age of 28 years. All tumors were confined to one ovary. Four tumors were well differentiated, 14 were intermediately differentiated, 20 were poorly differentiated, and 2 were undefined; 2 cases had heterologous elements, and 3 had a retiform pattern. The patients were classified into 3 groups: androgen excess (25/40), estrogen excess (6/40), and no endocrine changes (9/40). The percentages of tumors > 10 cm in diameter were 8.0%, 16.7% and 40.0%, respectively; the percentages of poor differentiation were 40.0%, 50.0% and 77.8%, respectively; and the percentages of tumor rupture were 20.0%, 16.7% and 66.7%, respectively. One patient underwent cystectomy, 27 underwent unilateral salpingo-oophorectomy, and 12 underwent total hysterectomy and bilateral salpingo-oophorectomy. A total of 23 patients received postoperative chemotherapy. One patient died of diabetic nephropathy, and 3 were lost to follow up. The remaining 36 were followed up from 12 to 377 (average 70.4) months. Two patients with stage Ic tumors of poor differentiation had a recurrence within 13 and 21 months, and both obtained complete remission after the second surgery and chemotherapy.

Conclusions

The prognosis of SLCTs is good, although poorly differentiated tumors may recur. Conservative surgery is acceptable for young patients wishing to preserve fertility, and postoperative adjuvant chemotherapy and long-term follow up are recommended to those with high-risk factors.     

Treatment of severe androgen excess due to ovarian hyperthecosis with a long-acting gonadotropin-releasing hormone agonist

A 31-year-old nulligravid patient presented with irregular menses, severe hirsutism, and infertility. Evaluation revealed marked increases of serum androstenedione and testosterone levels and a possible ovarian mass. At operation a cystic teratoma was removed from the left ovary and bilateral wedge resection revealed severe ovarian hyperthecosis. After operation only a transient decrease of androstenedione and testosterone was noted and the patient failed to ovulate or improve clinically. Subsequently a long-acting gonadotropin-releasing hormone agonist was administered daily for 6 months, which reduced circulating delta 4-steroids and estrogens to levels approximating those of castrated women. Immediately after discontinuation of treatment, ovulation induction was successfully achieved with human menopausal gonadotropin. This report introduces a new therapeutic approach to the problem of severe ovarian hyperthecosis and may provide an opportunity for childbearing in these patients.     

Cardiac tamponade due to ovarian carcinoma

Summary We describe a patient with ovarian carcinoma who presented with cardiac tamponade with subsequently development of cardiac arrest as the initial symptom. After successful resuscitation and pericardiocentesis our patient was given an intrapericardial infusion of bleomycin and there was no recurrence of pericardial effusion at 14 months follow-up.     

Bilateral ovarian lipid cell hyperplasia in a young hirsute patient

Virilizing lipid cell ovarian hyperplasia occurrd in a 24-year-old woman referred with secondary amenorrhea, hirsutism, and weight gain. Hormone analyses revealed abnormal androgen production shown in a suppression test to be autonomic and of ovarian origin.Ovarian vein catheterization revealed abnormally increased androgen levels in the left ovarian vein and in the caval vein where it was entered by the right ovarian vein. As the patient wanted to become pregnant, three-quarters of her ovarian tissue was resected. Menstruation and ovulation returned. Two years after the operation the patient conceived.Histopathological and electron microscopic examinations revealed bilateral lipid cell hyperplasia.     

Peripheral Precocious Puberty due to Functioning Adrenocortical Tumor: Description of Two Cases

Background

Adrenocortical tumors (ACTs) represent less than 0.2% of all childhood neoplasms. Frequent clinical manifestations are virilization, hypercortisolism, and peripheral precocious puberty (PPP).

Postmenopausal bleeding due to chronic DIC

'Bleeding disorders' are listed as a cause of postmenopausal bleeding even though there appear to be no reported cases. We present a case of postmenopausal bleeding, because of chronic DIC, which resulted from aortic aneurysm and was managed with replacement blood products and tranexamic acid.     

Steroid and gonadotropin secretion in a patient with a 30-year history of virilization due to lipoid-cell ovarian tumor

A 36-year-old woman with a 30-year history of undiagnosed virilizing lipoid-cell ovarian tumor is described. The tumor was localized by the NP-59 scan. Data of extensive investigations of steroid and gonadotropin secretion are provided. The preoperative dynamics of serum dehydroepiandrosterone sulfate and its decrease after the removal of the tumor suggested that dehydroepiandrosterone sulfate was being secreted by the ovarian tumor. Ovarian-peripheral venous gradients for plasma delta 5 and delta 4 steroids were correlated with the in vitro measurements of various steroidogenic enzymatic activities in the tumor tissue. The proposed metabolic pathway of the tumor is as follows: Pregnenolone----17-hydroxypregnenolone----dehydroepiandrosterone---- androstenedione----testosterone----estradiol. Low basal gonadotropin levels with no discernible pulsatility and no response to gonadotropin-releasing hormone were observed preoperatively, and a gradual normalization in both parameters was observed during the six months after removal of the tumor.     

Maternal virilization in pregnancy due to an unclassified sex-cord stromal neoplasm

Virilization in pregnancy has been reported with various ovarian neoplasms. Presented is a case of maternal virilization resulting from an unclassified sex-cord stromal neoplasm.     

Intervenciones dietéticas en el tratamiento del síndrome del ovario poliquístico. Una revisión bibliográfica

Polycystic ovary syndrome (PCOS) is a female endocrinopathy recognized as a heterogeneous disorder characterized by hyperandrogenism and ovulatory dysfunction that leads to fertility problems. In addition, patients usually present with associated symptoms such as insulin resistance, glucose intolerance, central obesity and/or metabolic syndrome that can induce an increased risk of cardiovascular disease. Since one of the main goals of PCOS is to reduce the metabolic consequences related to obesity, insulin resistance, and the metabolic syndrome, targeted dietary interventions may be effective in treating PCOS.A bibliographic search has been carried out in different databases such as Web of Science, Pubmed and Google Scholar, establishing previously defined search criteria. Eleven have been chosen for full review and critical analysis. Among the different interventions that have been used, dietary strategies have been followed such as the dietary approaches to stop hypertension (DASH), modifications in carbohydrates, the inclusion of a certain food in the usual dietary pattern and/or lifestyle modifications. Of the results obtained, we highlight the improvements in body markers with a DASH diet, the benefits promoted by diets with modifications in carbohydrates, in insulin resistance and hormonal markers and favorable effects on clinical manifestations related to hyperandrogenism, fostered by soy consumption and lifestyle modifications.     

Surgical management of infertility due to polycystic ovarian syndrome after failure of medical management

Objectives

To evaluate surgical management and fertility and pregnancy outcome in women with polycystic ovarian syndrome (PCOS).

Study design

Retrospective file review and follow-up of 74 consecutive women with PCOS resistant to citrate clomiphene (CC) who underwent ovarian drilling by fertiloscopy with bipolar energy, together with hysteroscopic surgery when indicated (Canadian TASK FORCE II-2).

Results

Of 77 files, only 3 women were lost to follow-up. Mean age was 30.2 years (SD 5.3) [29.0–31.4 CI 95%], and mean BMI 25.6 kg/m² (SD 6.2) [24.2–27.0 CI 95%]. Pregnancy occurred after drilling in 47 cases (63%), spontaneously in 20 (27%), after ovarian stimulation in 5 (6.7%) and after in vitro fertilization in 22 (29.7%). Laparoscopic conversion was required in 5 cases (6.7%), due to failure to visualize the adnexa (n = 3), or pelvic adhesions (n = 1), or uterine hemorrhage (n = 1). Hysteroscopy detected and simultaneously treated a uterine anomaly in 18 of 74 patients: uterine septum (n = 10, 13%), T-shaped uterine cavity (n = 3, 4%), endometrial polyp (n = 2, 2.7%), endometrial hypertrophy (n = 2, 2.7%), and synechiae (n = 1, 1.3%).The mean overall delay to pregnancy was 11.1 months (SD 8.5) [8.7–13.5 CI 95%] and to spontaneous pregnancy, 7 months (SD 7.6) [3.7–10.3 CI 95%]. The mean follow-up was 23.4 months (SD 16.5) [18.1–28.7 CI 95%]. After multivariate analysis, the likelihood of pregnancy was significantly associated with previous ovarian stimulation by FSH (OR = 2.28, 95% CI = 1.08–4.83) and initial FSH level (OR = 0.52, 95% CI = 0.29–0.93).

Conclusion

Ovarian drilling by hydrolaparoscopy is an effective treatment for CC-resistant PCOS. The high rate of associated uterine anomalies justifies simultaneous hysteroscopic surgery.