1/13. Ovarian hyperthecosis in the setting of portal hypertension.Hepatocellular dysfunction and perturbed portal hemodynamics alter steroid metabolism. Men with liver disease have gynecomastia, although women similarly affected rarely show virilization. We report a 10-yr-old girl with portal hypertension and shunting associated with precocious puberty and ovarian hyperandrogenism. This was one of premature twin girls; neither had clitoromegaly or genital ambiguity. In one child, neonatal respiratory problems led to umbilical vein catheterization with subsequent development of portal hypertension. Pubic hair was first noted at age 6 yr, breasts at 7 yr, and severe acne and clitoromegaly at 10 yr. Baseline sex hormones were elevated: androstenedione (A), 413 ng/dL; testosterone (T), 226 ng/dL; and estradiol (E2), 160 pg/mL. liver transaminases were within the normal range, however, the coagulation profile was mildly abnormal. cosyntropin adrenal stimulation revealed no steroidogenic defect. dexamethasone suppression reduced A and T slightly. LH-releasing hormone stimulation produced a pubertal rise in LH and FSH. Pelvic sonography showed a large right ovary with numerous follicles. Surgical exploration revealed symmetrically enlarged ovaries with dense capsules. histology of ovarian wedge resections showed hyperthecosis; immunohistochemistry showed stromal cells expressing steroidogenic enzymes and proteins. One month postoperatively, A and T were unchanged from baseline, whereas E2 decreased to 56 pg/mL. A single dose of depot leuprolide acetate significantly reduced T. Subsequent treatment with oral contraceptives reduced T to 50 ng/dL, and cyclical menses occurred. We conclude that precocious puberty and ovarian hyperthecosis were induced in this young girl by elevated circulating levels of sex hormones, a consequence of portasystemic shunting and impaired hepatic steroid metabolism.- - - - - - - - - - ranking = 1keywords = androgen (Clic here for more details about this article) |
2/13. Long-term remission of ovarian hyperandrogenism after short-term treatment with a gonadotropin-releasing hormone agonist.OBJECTIVE: To assess the long-term effects of GnRH agonist (GnRH-a) therapy in a patient with benign ovarian hyperandrogenism. DESIGN: Case report. SETTING: University Hospital endocrine outpatient's clinic. PATIENT(S): A 55-year-old postmenopausal woman with hirsutism and virilization of ovarian origin. INTERVENTION(S): Treatment with a course of GnRH-a (triptorelin 3.75 mg IM every 28 days for 4 months). Follow-up for 3 years. MAIN OUTCOME MEASURE(S): serum gonadotropin and androgen levels, clinical assessment using the Ferriman-Gallwey score, and assessment of ovarian morphology by ultrasonography. RESULT(S): Administration of triptorelin resulted in suppression of serum testosterone and gonadotropin values and relief of the hyperandrogenic symptoms. Upon discontinuation of treatment, the patient's serum gonadotropin levels returned to the postmenopausal range, but the testosterone levels remained normal and the patient was asymptomatic for an observation period of 3 years. CONCLUSION(S): This case is the first example of long-term remission of ovarian hyperandrogenism in a postmenopausal woman, after short-term treatment with GnRH-a. This supports the view that GnRH-a therapy could be used, even in short courses, for the long-term suppression of benign ovarian hyperandrogenism.- - - - - - - - - - ranking = 9keywords = androgen (Clic here for more details about this article) |
3/13. The gonadotropin-resistant ovary syndrome in association with secondary amenorrhea.A young patient with secondary amenorrhea and primary infertility is described. After a gynecological-endocrinological exploration including laparoscopy, the diagnosis indicates secondary hypergonadotropic, hypo-estrogenic normo-androgenic amenorrhea. The anatomopathological examination of an ovarian biopsy revealed an intact follicular apparatus, thus disproving the suspected diagnosis of climacterium praecox. Since very high gonadotropin doses could not induce an ovulation, it was concluded that the rare combination of secondary amenorrhea and the gonadotropin-resistant ovary syndrome must be present. The pathogenesis of this syndrome is discussed.- - - - - - - - - - ranking = 1keywords = androgen (Clic here for more details about this article) |
4/13. Peripheral and Ovarian steroids in Ovarian Hyperthecosis.serum levels of cortisol (F), pregnenolone (delta5-P), 17-hydroxypregnenolone (17-delta5-P), progesterone (P), 17-hydroxyprogesterone (17-P), androstenedione (A), testosterone (T), 5alphadihydrotestosterone (DHT), dehydroepiandrosterone (DHEA), its sulfate (DHEA-S), estrone (E1), and estradiol-17beta (E2) were measure in 2 virilized patients with ovarian hyperthecosis. Daily morning blood samples were obtained for 6 consecutive days. dexamethasone (Dex) 2 mg/day was administered orally starting after venipuncture on the second day and continued for 5 days. Human chorionic gonadotropin (hCG) was administered intramuscularly on the afternoon of the fourth and fifth days. Following the suppression-stimulation test, both patients underwent abdominal hysterectomy and bilateral salpingo-oophorectomy. At the time of surgery, samples of peripheral and ovarian vein blood were obtained for steroid measurements. Blood samples were also obtained postsurgery to evaluate the effect of ovariectomy on the steroid levels. Of significance were the following observations: 1)Although both patients were eumenorrheic, no corpus luteum or corpus albicans was seen on histologic examination of the ovaries. 2)Of the androgens measured, only peripheral T and DHT were elevated and did not suppress on Dex treatment, but decreased to low levels following ovariectomy, pointing toward the ovary as the source of excess T and DHT. Both patients had elevated T and DHT in the ovarian vein samples. 3)In 1 patient the ovarian vein samples shoed elevated F levels with a significant ovarian-peripheral venous gradient for this steroid, and indication of ovarian secretion of F in this patient. 4)The levels of 17-P were elevated in both patients, did not suppress on Dex, and increased markedly following hCG, suggesting the ovary as the source of excess 17-P. Since A levels were normal and did not increase concomitantly with 17-P levels following hCG, it is likely that the patients had a decreased activity of the ovarian C17-20 desmolase, the enzyme responsible for the conversion of 17-P to A.- - - - - - - - - - ranking = 1keywords = androgen (Clic here for more details about this article) |
5/13. Virilization during pregnancy due to bilateral hyperthecosis. A case report.A case of recurrent maternal virilization associated with bilateral hyperthecosis of the ovary during two pregnancies is described. In the first pregnancy, serum testosterone increased to the level of 12.5 nmol/l and in the second pregnancy to 35 nmol/l and after delivery to 66 nmol/l.5 months after the second birth the testosterone level was 2.7 nmol/l and virilization had spontaneously regressed. Puerperal stimulation tests with human menopausal or with human chorionic gonadotropin did not increase ovarian androgen production. female babies were not virilized.- - - - - - - - - - ranking = 1keywords = androgen (Clic here for more details about this article) |
6/13. Fibromatosis and massive edema of the ovary, possibly related entities: a report of 14 cases of fibromatosis and 11 cases of massive edema.Twenty-five cases of ovarian enlargement secondary to massive edema, to a hitherto undescribed lesion that we have designated fibromatosis, or to both processes were reviewed to explore the possibility of a relation between them. The patients ranged in age from 13 to 39 years. The 14 patients with pure or predominant fibromatosis usually presented because of menstrual abnormalities; two had evidence of androgen excess and three had abdominal pain. At laparotomy two involved ovaries were observed to have twisted on their pedicles. There was definite bilateral ovarian involvement in two cases and probable bilateral involvement in two others. The excised specimens measured up to 12 cm in diameter and typically had firm, white sectioned surfaces. Microscopic examination showed a proliferation of spindle cells usually separated by dense collagen, which surrounded normal follicular structures. Lutein cells were present in the fibromatous areas in one patient, who was masculinized, and in the adjacent ovarian stroma in another patient, who was hirsute; a proliferation of cells of sex cord type was observed in the fibromatous areas in three cases. Six of the specimens contained focal areas of edema similar in appearance to that seen in cases of massive edema. The features of the 11 cases of massive edema were, in general, similar to those of the 40 examples of this entity reported in the literature. Six of the 11 patients had menstrual abnormalities and/or evidence of androgen excess, but most of them presented because of abdominal pain. At operation an involved ovary was observed to have undergone torsion in five of the cases; bilateral ovarian enlargement due to massive edema was present in two cases. The excised specimens, which measured up to 15 cm in diameter, typically had a watery appearance on sectioning. Microscopic examination showed edematous stroma surrounding residual normal ovarian structures. Lutein cells were identified in the edematous stroma in four cases and in the adjacent ovarian stroma in three of these cases. One of these patients was masculinized and another was hirsute. Small foci of fibromatosis similar to that seen in cases of pure fibromatosis were present in eight cases. The similar age range and clinical manifestations of these two processes and the overlap in their histologic features suggest that they are closely related and may reflect differing morphologic expressions of the same underlying disorder. Some of the cases of massive edema, however, may result from the development of stromal edema in ovaries involved by hyperthecosis.- - - - - - - - - - ranking = 2keywords = androgen (Clic here for more details about this article) |
7/13. Ovarian hyperthecosis: in vivo and in vitro correlations of the androgen profile.A 36-year-old patient with secondary amenorrhea and progressive frank virilization was found by histologic examination to have ovarian hyperthecosis. Baseline androgenic investigation revealed a selective increase in testosterone (T) production rate (3.1 mg/day), which was associated with an increase in both plasma T concentration (total and unbound) and metabolic clearance rate (MCR) of T (2156 liters/day). There was a marked concentration gradient for circulating plasma T between each ovarian vein and the peripheral veins (right ovarian, 50 ng/ml; left ovarian, 21 ng/ml). After bilateral wedge resection there was a rapid decrease in the total and unbound T levels as well as in the MCR of T, which remained normal for at least 6 months. By then the patient had stopped shaving and was menstruating normally. in vitro incubation studies of fragments from each ovary revealed incorporation of [3H]-dehydroepiandrosterone and [3H]-progesterone into [3H]-T in the range reported for normal human ovarian stroma, and short-term cellular culture suggested a normal morphology and steroidogenesis. It is concluded that in this patient with ovarian hyperthecosis, the observed ovarian T production resulted more from an increase in the amount of stromal-thecal tissue than from a functional biochemical defect in the latter.- - - - - - - - - - ranking = 5keywords = androgen (Clic here for more details about this article) |
8/13. Massive edema of the ovary associated with androgenic manifestations.Massive ovarian edema is a rare tumor-like condition of the ovary characterized by marked enlargement of one or both ovaries due to marked accumulation of edema fluid in the ovarian stroma. This paper reviews the literature on massive ovarian edema and presents a case associated with androgenic manifestations.- - - - - - - - - - ranking = 5keywords = androgen (Clic here for more details about this article) |
9/13. Massive ovarian edema with androgen secretion. A pathological and endocrine study with review of the literature.A case of massive edema of the left ovary with virilization is described. Microscopically, massive interstitial edema with luteinization of theca and stromal cells was found. A few stromal cells contained Reinke-type crystalloids--an original observation. Peripheral concentrations of testosterone, dihydrotestosterone and androstenedione were increased. Ratios of left ovarian vein to peripheral vein concentrations were increased for all these steroids as well as for estradiol and estrone, showing that the left ovary was the source of excess androgen and estrogen secretion. The patient showed impaired gonadotropin secretion in basal conditions and after an intravenous luteinizing-hormone-releasing hormone (LHRH) stimulation test. After left oophorectomy, all steroids and gonadotropin response to LHRH returned to normal, and virilization regressed. Analysis of the endocrine changes associated with this ovarian tumor brings additional arguments for a primary role of hyperandrogenism in the impairment of gonadotropin secretion, as was also observed in other hyperandrogenic disorders including polycystic ovarian syndrome.- - - - - - - - - - ranking = 7keywords = androgen (Clic here for more details about this article) |
10/13. Reduction of insulin resistance after correction of nonneoplastic ovarian virilization.A 60-year-old woman with a progressive virilization for about 5 yr developed diabetes mellitus with elevated insulin levels (fasting insulinemia ranging 32.4-45.8 microU/ml). The marked increase of plasma testosterone (total 5.7-8.2 ng/ml; free 11.5 pg/ml) and other endocrine markers clearly indicated the ovarian origin of hyperandrogenism. Pelvic ultrasonography, computerized axial tomography, and direct examination of ovaries during laparotomy, showed no evidence of neoplasia. Microscopic examination and immunocytochemical investigations confirmed the diagnosis of hyperthecosis. After oophorectomy and regression of hyperandrogenism, fasting and postprandial blood glucose concentrations normalized in spite of persistently elevated levels of insulinemia (fasting values ranging 32.0-61.0 microU/ml). The present case suggests that pathological increase of testosterone can interfere with insulin-glucose balance impairing the peripheral sensitivity to insulin.- - - - - - - - - - ranking = 2keywords = androgen (Clic here for more details about this article) |
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