1/12. Diffuse stromal Leydig cell hyperplasia: a unique cause of postmenopausal hyperandrogenism and virilization.A 60-year-old woman presented with diffuse scalp alopecia, hirsutism, and clitorimegaly, and the mean serum testosterone levels were greater than 200 ng/dL. Findings on computed tomography of both adrenal glands were normal. After bilateral oophorectomy, a unique histological picture consisting of diffuse stromal Leydig cell hyperplasia was found. Reinke crystals were present, but neither hilus cell hyperplasia nor stromal hyperthecosis was noted. Sequencing of the 11 exons of the gene for the luteinizing hormone receptor revealed no abnormality. Relevant data suggest that treatment of the postmenopausal woman with hyperandrogenism and virilization is bilateral laparoscopic oophorectomy if she has no pronounced ovarian enlargement or adrenal tumor on imaging. In this setting, an intensive endocrine evaluation or a search for metastatic disease seems to be unnecessary.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
2/12. Postmenopausal virilization, simple ovarian cyst, and hilus cell hyperplasia--is there an association?OBJECTIVE: To report a case of virilizing ovarian hilus cell hyperplasia detected postmenopausally in association with a simple cyst and to review the related literature, including four similar cases. methods: Hormonal and pathologic studies were conducted, and ovarian venous catheterization was performed during total abdominal hysterectomy. RESULTS: In our 69-year-old female patient, serum testosterone levels were 508, >3,200, and 11 ng/dL, respectively, in peripheral blood preoperatively, in ovarian venous blood obtained intraoperatively, and in peripheral blood postoperatively. The wall of the cyst contained several clusters of hilus cells, which were also found asymmetrically lateralized to the affected ovary. CONCLUSION: Hilus cell hyperplasia should be suspected in any case of postmenopausal virilization in which ultrasonography or magnetic resonance imaging suggests the presence of a simple ovarian cyst.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
3/12. Ovarian mucinous cystadenocarcinoma with virilization.BACKGROUND: ovarian neoplasms, other than sex cord--stromal tumors, are rare causes of hyperandrogenism. Only two cases of primary mucinous carcinomas associated with virilization have been reported. CASE: A 50-year-old female was referred to our clinic with a large pelvic mass. On examination she had significant facial hirsutism, clitoromegaly, and male pattern pubic hair growth. serum levels of testosterone and dihydroepiandrosterone sulfate were elevated. A 30-cm, multilocular, solid and cystic, left ovarian mass was resected. histology revealed moderately to poorly differentiated mucinous cystadenocarcinoma. The ovarian stroma contained florid proliferation of luteinized cells. The right ovary showed cortical stromal hyperplasia. Abnormal hormone values normalized 10 days postoperatively. CONCLUSION: We report a rare case of mucinous cystadenocarcinoma with virilization, review the literature, and discuss the mechanisms of hormone production by these tumors.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
4/12. Treatment with flutamide decreases cortisol clearance: implications for therapy in congenital adrenal hyperplasia.BACKGROUND: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by a defect in cortisol and often aldosterone secretion, and adrenal hyperandrogenism. Current treatment is to provide adequate glucocorticoid and mineralocorticoid substitution to prevent adrenal crises and to suppress excess adrenocortical androgen secretion. Anti-androgen therapy with flutamide is an option that allows control of hyperandrogenism without recourse to supraphysiological doses of glucocorticoid. methods: We examined the pharmacokinetic parameters of hydrocortisone administered i.v. as a bolus at a dose of 15 mg/m2 in a 17.3 year-old female patient with classic CAH before and four weeks after institution of flutamide treatment by determining serum cortisol concentrations at 10 min intervals for 6 h following the i.v. bolus of hydrocortisone. RESULTS: Treatment with flutamide resulted in a decrease in cortisol clearance from 420 ml/l to 305 ml/l (27% reduction), and a decrease in volume of distribution from 51.61 to 451 (12.9% reduction). The half-life of cortisol increased from 85.3 min to 102.1 min. CONCLUSIONS: flutamide treatment decreases cortisol clearance, thereby prolonging its half-life. These findings indicate that a reduction in the daily dose of glucocorticoid replacement may need to be considered when flutamide is added to the treatment regimen of patients receiving hydrocortisone.- - - - - - - - - - ranking = 0.83333333333333keywords = hyperplasia (Clic here for more details about this article) |
5/12. Virilization in bilateral macronodular adrenal hyperplasia controlled by luteinizing hormone.We report a case of a virilized 59-yr-old woman with elevated serum testosterone levels and bilateral macronodular adrenal hyperplasia. The patient underwent laparoscopic right adrenalectomy, after which the elevated testosterone level transiently normalized. The immediate postoperative depression of the testosterone level suggested that the process was driven by gonadotropins that were suppressed by the stress of surgery. The excised right adrenal mass contained testosterone by immunohistochemistry and LH receptor mRNA by in situ hybridization. The recurrence of hyperandrogenemia suggested that the enlarged left adrenal was also secreting testosterone. The serum testosterone level increased in response to im injection of human chorionic gonadotropin, suggesting control by aberrant LH receptors. Injection of leuprolide acetate (7.5 mg im) to suppress LH levels resulted in normalization of the testosterone level 12 d later that persisted for several weeks. Ectopic receptors mediating Cushing's syndrome have been described in several cases of bilateral adrenal hyperplasia and adrenal adenoma. This is the first case to our knowledge in which pure androgen overproduction in adrenal hyperplasia has been shown to be controlled by LH receptors. In our patient, the control of androgen secretion by LH may explain the postmenopausal onset of virilization and the transient postoperative normalization of the serum testosterone level.- - - - - - - - - - ranking = 1.1666666666667keywords = hyperplasia (Clic here for more details about this article) |
6/12. Seckel-like syndrome: a patient with precocious puberty associated with nonclassical congenital adrenal hyperplasia.Seckel syndrome is a rare, recessively inherited disorder of severe growth retardation with low birth weight and distinct craniofacial, orodental and skeletal anomalies. In addition to these characteristics, some cardiovascular, hematopoietic, endocrine and central nervous system abnormalities have also been described. We report a patient with Seckel-like syndrome who has precocious puberty associated with non-classical congenital adrenal hyperplasia (NCCAH). She was admitted to our clinic three times. She was diagnosed as having Seckel-like syndrome and premature thelarche at the age of 8.9 years. At 10.9 years old she was admitted to our clinic with pubic hair and cliteromegaly. Hormonal findings revealed NCCAH and hydrocortisone therapy was offered but the patient was non-compliant. At 13.6 years she had acanthosis nigricans as an additional clinical finding and her pubertal stage was 4. She had irregular menses. On hormonal evaluation she had euglycemic hyperinsulinism accompanying mild hypertriglyceridemia and functional ovarian hyperandrogenism. Premature pubarche, hyper-insulinism, dyslipidemia, and hyperandrogenism, and some combinations of these, can be associated with reduced fetal growth. This is the first report of hyperinsulinism, and probably NCCAH, in association with Seckel syndrome.- - - - - - - - - - ranking = 0.83333333333333keywords = hyperplasia (Clic here for more details about this article) |
7/12. The case of the elusive androgen.OBJECTIVE: To describe a case of androgen excess and discuss the important factors in diagnosis and management. methods: A case report is presented of a postmenopausal woman who had had severe hirsutism for 18 months. Her history, clinical and laboratory findings, treatment, and outcome are chronicled. The pertinent literature--especially that related to the differential diagnosis of hyperandrogenism--is also reviewed. RESULTS: A 62-year-old woman had progressive hirsutism of the face, back, and abdomen as well as alopecia of the scalp, for which spironolactone therapy had proved ineffective. Laboratory studies showed a testosterone level of 644 ng/dL. Preoperative evaluation pointed to an ovarian source of testosterone. After total abdominal hysterectomy and bilateral oophorectomy, histologic examination of the ovaries showed bilateral hilar cell hyperplasia. Three months later, the serum testosterone level remained high (556 ng/dL), and repeated computed tomography of the abdomen disclosed a previously unseen 9-mm adenoma of the left adrenal gland, which was removed laparoscopically. Because of a persistently high testosterone value (546 ng/dL), the patient underwent dexamethasone suppression studies, followed by adrenal stimulation with corticotropin; no pathologic findings were demonstrated. Finally, gonadotropin suppression with nafarelin, 200 mg intranasally daily for 6 weeks, yielded a prompt and sustainable decrease in the testosterone level. This result was associated with dramatic clinical improvement. CONCLUSION: It is speculated that the patient had residual testosterone-producing tissue originating from primitive mesenchymal cells from the urogenital ridge, which was responsive to gonadotropins, in an unidentified abdominal or pelvic site.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
8/12. Mild cutaneous manifestation in two young women with extraordinary hyperandrogenemia.hyperandrogenism with hyperandrogenemia should be considered in those with severe acne of sudden onset or conspicuous male-pattern baldness with hairline recession, although the majority of female patients with acne or androgenetic alopecia possess no endocrine disorder. Herein we describe on the contrary 2 young women with primary amenorrhea displaying prominent hyperandrogenemia but subtle cutaneous manifestation. The first one presenting vertical alopecia had an elevated level of serum dehydroepiandrosterone sulfate (>800 microg/dl) and was suspected to be a case of late-onset, non-classical adrenal hyperplasia. The second case with mild acne had a soaring serum level of total testosterone >9,000 ng/dl derived from an androgen-secreting adrenal adenoma overexpressing steroidogenic acute regulatory protein, P450 side-chain cleavage enzyme and aromatase. A careful patient history and a complete physical examination are mandatory in each individual female case with acne or alopecia. The possibility of adrenal tumor should be explored in patients with escalated circulating testosterone.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
9/12. Paraadrenal Castleman disease presenting with adrenal hyperandrogenism.Castleman disease, or angiofollicular lymph node hyperplasia, is a rare benign lymphoproliferative disorder. We report an uncommon case of retroperitoneal Castleman syndrome associated with adrenal hyperandrogenism and with interleukin-6 as the possible link in the pathophysiology of both disturbances. Four years after surgical resection of the paraadrenal mass, the patient is free of signs of recurrence of Castleman disease and adrenal hyperandrogenism.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
10/12. hyperandrogenism due to 3 beta-hydroxysteroid dehydrogenase deficiency with accessory adrenocortical tissue: a hormonal and metabolic evaluation.1. Adrenal ectopic tissue has been detected in the paragonadal region of normal women. In patients with congenital adrenal hyperplasia due to 21-hydroxylase (21-OH) deficiency, the manifestation of hyperplasia of paragonadal accessory adrenal tissue has been usually reported to occur in males. Probably, this is the first report of a female with 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency with ectopic adrenal tissue in ovaries. However, the occurrence of hyperplasia of adrenal rests among women with classical congenital adrenal hyperplasia may not be rare, especially among patients with a late diagnosis. 2. We report a woman with 3 beta-HSD deficiency whose definitive diagnosis was made late at 41 years of age immediately before surgery for the removal of a uterine myoma. During surgery, exploration of the abdominal cavity revealed the presence of bilateral accessory adrenal tissue in the ovaries and in the para-aortic region. The patient had extremely high levels of ACTH (137 pmol/l), DHEA (901.0 nmol/l), DHEA-S (55.9 mumol/l), androstenedione (70.2 nmol/l), testosterone (23.0 nmol/l) and 17 alpha-hydroxypregnenolone (234.4 nmol/l) suggesting 3 beta-HSD deficiency. 3. In view of these elevated androgen levels, with an absolute predominance of DHEA and DHEA-S, we evaluated the effect of this hormonal profile on carbohydrate tolerance and insulin response to glucose ingestion. 4. The patient presented normal glucose tolerance but her insulin response was lower than that of 14 normal women (area under the curve, 3 beta-HSD = 17,680 vs 50,034 pmol/l for the control group over a period of 3 h after glucose ingestion).(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 0.66666666666667keywords = hyperplasia (Clic here for more details about this article) |
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