Cases reported "Ovarian Cysts"

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1/17. Hyperreactio luteinalis despite the absence of a corpus luteum and suppressed serum follicle stimulating concentrations in a triplet pregnancy.

    Hyperreactio luteinalis is characterized by moderate to marked cystic enlargement of the ovaries related to multiple theca lutein cysts and is associated with very high sex steroid concentrations. It is a rare condition especially in the first trimester. The case described below is believed to be the only case of hyperreactio luteinalis reported following frozen embryo transfer. This case provides an opportunity to gain further insight into the mechanism responsible for this unusual condition. The 30 year old woman demonstrated a slightly elevated LH/FSH ratio (5 and 3 mIU/ml respectively) and normal baseline androgen concentrations. Two years following oocyte retrieval she had a second frozen embryo transfer. The ovaries were normal size when the embryos were transferred and androgens were still normal. The ovaries did not begin to enlarge until 51 days from transfer. A dichorionic intrauterine pregnancy with monozygotic twins in the left gestational sac was seen. Eventually, 86 days from transfer, the ovaries enlarged to 145x103x116 mm right; and 83x95x117 mm left. serum oestradiol was 30 078 pg/ml, beta-human chorionic gonadotrophin (HCG) 239 920 mIU/ml, serum progesterone >160 ng/ml, total testosterone 2254 ng/dl, free testosterone 42.3 pg/ml and androstenedione 7328 ng/dl. Throughout the first trimester, serum FSH was <1 mIU/ml. Thus, neither FSH nor a corpus luteum is necessary to initiate this syndrome.
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2/17. Ovarian cyst in a premature infant treated with spironolactone.

    We describe the late occurrence of an ovarian cyst in a premature infant, the second of a set of monochorionic diamnionic twins, during treatment with spironolactone. spironolactone is commonly used in the management of neonatal chronic lung disease in combination with other diuretics because of its potassium-sparing effect. It has progestational activity and has been reported to cause gynecomastia. It is used widely for its antiandrogenic properties in the treatment of female hirsutism and hyperandrogenism and it has a role in the management of familial male-limited precocious puberty. However, the estrogenic influence induced during therapy may significantly alter the hormonal milieu in newborn females, resulting in the formation of ovarian cysts. Simple ovarian lesions <5 cm merit close ultrasonographic surveillance for resolution while larger complex masses may warrant surgical intervention.
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3/17. The role of LH and FSH in ovarian androgen secretion and ovarian follicular development: clinical studies in a patient with isolated FSH deficiency and multicystic ovaries.

    Inactivating mutations have proven to be instructive in elucidating the role of FSH in human ovarian function. We performed a detailed reproductive endocrine evaluation of a patient with inactivating mutations in the FSH beta-subunit gene who was hypo-estrogenic and had LH excess. The patient underwent a pelvic ultrasound and overnight frequent blood sampling followed by a human chorionic gonadotrophin (HCG) stimulation test. One month later she received human recombinant FSH, followed 24 h later by a second HCG stimulation test. Despite a mean LH serum concentration and LH pulse characteristics typical for polycystic ovaries (PCOS), baseline and dexamethasone-suppressed free testosterone were low-normal. The administration of HCG led to minimal stimulation of 17-hydroxyprogesterone and androgens. The patient had multicystic ovaries containing follicles 3-5 mm in diameter and responded to FSH with prompt increases in estradiol and inhibin B. There were no clinical or laboratory consequences of LH excess in this FSH-deficient woman. These findings support the hypothesis that excessive LH stimulation alone does not cause ovarian hyperandrogenism. We also found that follicular development was present in the absence of FSH. These antral follicles had apparently developed normally, since estradiol and inhibin B increased promptly after FSH administration.
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4/17. Deficiency of 17,20-lyase causing giant ovarian cysts in a girl and a female phenotype in her 46,XY sister: case report.

    A 13-year-old girl was referred because of progressive abdominal pain caused by ovarian torsion and giant ovarian cysts. Secondary sexual characteristics were absent. Hormone analysis revealed markedly elevated serum levels of progesterone and 17-hydroxyprogesterone in combination with very low peripheral concentrations of C19 steroids (dehydroepiandrosterone and androstenedione) and estrogens. serum concentrations of FSH and LH exceeded the upper limit of normal levels in adult women. The patient's 16-year-old 46,XY sibling showed a female phenotype with similar hormonal disturbances. Both siblings were found to be compound heterozygotes for two mutations in the CYP17 gene: an R347C mutation in one allele and a 25-base pair deletion in exon 1 in the other. The resulting block in 17,20-lyase activity caused an inability to synthesize androgens and estrogens, and increased levels of gonadotrophins due to a lack of negative feedback. The increased levels of gonadotrophins most likely stimulated growth of the ovarian cysts. The administration of a GnRH antagonist reduced the size of the cysts within a few weeks. At present, the girl is being treated with a combination of a GnRH agonist and hormone replacement therapy.
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5/17. Bilateral sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome.

    Thirty-year-old woman with lower abdominal pain was operated due to adnexial mass. cystectomy on right gonad revealed sertoli cell adenoma and simple serous cyst and left gonadal biopsy showed immature testis tissue. Later, laparoscopic left gonadectomy was made. Histopathology of the left gonad was consistent with sertoli cell adenoma.
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6/17. Steroid hormones in ovarian vein and cyst fluid of a virilizing stromal tumor.

    A 23-year-old female with clinical signs of excessive androgen and estrogen production was found to have a gonadal stromal tumor. The clinical symptoms correlated well with steroid hormone values in ovarian vein blood and ovarian cyst fluid of the ovarian tumor. Ovarian vein blood draining the ovarian tumor measured as follows: estrone, 162 pg/ml; estradiol, 1688 pg/ml; progesterone, 9.6 ng/ml; testosterone, 9775 ng/100 ml; and dihydrotestosterone, 2905 ng/100 ml. The ovarian cyst fluid produced higher estrogen and lower androgen values compared with ipsilateral ovarian vein blood. Peripheral levels of immunoreactive testosterone ranged from 210 to 244 ng/100 ml. It is concluded that excessive androgen and estrogen production may arise from a gonadal stromal tumor that has been previously classified as a purely virilizing ovarian tumor.
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7/17. Hyperreactio luteinalis in normal singleton pregnancy.

    A case of hyperreactio luteinalis in a patient with normal singleton pregnancy is reported. The course of pregnancy had been normal until the 24th week of gestation, when the mother developed lower abdominal pain and signs of virilization. She delivered of a normal female infant at 39 weeks' gestation. The baby did not show any signs of masculinization. serum testosterone, delta 4-androstene-dione, and 5 alpha-dihydrotestosterone of the mother were markedly elevated. They remained high after the delivery but returned to the normal ranges soon after the partial resection of the enlarged ovaries. Reported causes of hyperreactio luteinalis are reviewed. Their maternal serum androgen levels were compared with cases of luteoma of pregnancy.
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8/17. Abnormal steroid excretion in gestational trophoblastic disease complicated by ovarian theca-lutein cysts.

    serum and urine steroids were examined in two subjects with trophoblastic disease accompanied by large ovarian theca-lutein cysts and compared with those from 10 patients with trophoblastic disease but without palpable cysts. In the patients without cysts normal values were obtained for serum oestradiol, progesterone, 17 alpha-hydroxyprogesterone and androstenedione, and for urinary total oestrogens, pregnanediol, pregnanetriol, and 17-oxosteroids. Nineteen urinary steroid metabolites, quantified by capillary gas-liquid chromatography, were either within reference limits or marginally raised. In several cases relatively minor increases in serum testosterone and cortisol and urinary free cortisol were observed. In contrast, the subjects with cysts showed pronounced excesses of androgen metabolites, 17 alpha-hydroxypregnenolone, pregnanediol, and pregnanetriol, and both exhibited a similar pattern of unusual additional metabolites. The profiles superficially resembled those seen in 21-hydroxylase deficiency adrenogenital syndrome, but there were important discrepancies reflecting known differences in ovarian and adrenal steroid metabolism. Chemotherapy led to decline of human chorionic gonadotrophin concentrations, regression of the cysts, and return to normal of the steroid profile. Excess steroids in the patients with cysts may have originated in the ovary rather than in the trophoblastic tissue.
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9/17. Transient ovarian testosterone and androstenedione hypersecretion: a cause of virilization or premature pubarche in prepubertal girls.

    In 2 girls with signs of androgen overproduction, the usual causes were excluded. Patient 1 (3.6 years) presented with hypertrophy of the clitoris, patient 2 (7.8 years) with pubic and axillary hair. Urinary steroids and plasma dehydroepiandrosterone, 17-hydroxyprogesterone and estradiol were normal, but testosterone and androstenedione elevated in both cases. Echography showed polycystic ovaries. Testosterone and androstenedione returned to normal after laparotomy and removal of ovarian cysts in patient 1, and spontaneously in patient 2, in whom puberty started later appropriately for bone age.
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10/17. Endocrine studies in a pregnancy complicated by ovarian theca lutein cysts and hyperreactio luteinalis.

    Severe virilization developed in a pregnant woman in association with the occurrence of theca lutein cysts and hyperreactio luteinalis of the ovaries. At term, maternal serum levels of androstenedione (58 ng/mL) and testosterone (20 ng/mL) were elevated massively; estrone (24 ng/mL) and estradiol-17 beta (23 ng/mL) levels were increased moderately. Maternal serum levels of human chorionic gonadotropin (hCG) just before delivery, 22,276 mlU/mL, though twice the mean for normal women at term, were within the normal range. The levels of androstenedione (1.06 ng/mL) and testosterone (0.26 ng/mL) in umbilical cord serum of her normal newborn female infant were normal. The levels of androstenedione and testosterone in serum of this woman declined slowly, but progressively, during the first two months after delivery; the serum levels of androstenedione and testosterone increased substantially, however, after she was treated with hCG eight weeks postpartum. These results are suggestive that, for reasons unknown, markedly increased androgen production with theca lutein cysts and hyperreactio luteinalis in some pregnant women results ultimately because of increased ovarian sensitivity to hCG.
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