Cases reported "Hirsutism"

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1/16. Unilateral macronodular adrenal hyperplasia as an unusual cause of Cushing's syndrome--a case study.

    To appraise clinicians of the problems that may be encountered in the diagnosis and management of Cushing's syndrome, we present a case report of a 20-year old female, who was admitted with a recently developed central obesity, ammenorrhea hirsuitism, proximal myopathy and depression. She was found to have multiple striae, thin skin, elevated blood pressure glycosuria and hyperglycaemia. Morning and mid-night plasma cortisol concentrations revealed elevated levels, with a loss of diurnal variation. There was a failure of the normal suppressibility of cortisol secretion by low doses of dexamethasone, while a significant suppression of plasma cortisol concentration was observed with high doses of dexamethasone. There were no significant abnormalities observed in the pituitary fossa on skull radiograph and on the cranial computerised tomographic scan. After a period of stabilisation, she had a bilateral adrenalectomy done, with a histopathological finding of a left adrenal macronodular hyperplasia, while the right adrenal gland was small and friable. There was an uneventful post-operative period, with a gradual return to normality of most of the presenting complaints. The hyperglycaemia and hypertension got controlled without medications, while her menstrual cycles resumed within three months of bilateral adrenalectomy. This case report illustrates that an adrenal-dependent Cushing's syndrome may mimic a pituitary-dependent one, especially as regards the suppressibility of plasma cortisol secretion by high doses of dexamethasone.
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2/16. Use of a long-acting gonadotrophin-releasing hormone analogue in a postmenopausal woman with hyperandrogenism due to a hilus cell tumour.

    OBJECTIVE: The aim of this study was to prove the utility of GnRH analogues for the suppression of androgen secretion in a postmenopausal woman with a suspected virilizing ovarian tumour. DESIGN AND methods: We present a case of a 72-year-old woman with virilization of recent onset. Hormonal studies revealed a fourfold increase in serum testosterone levels, normal dehydroepiandrosterone sulphate concentrations and high levels of serum 17-hydroxyprogesterone levels. Computed axial tomography scan of the ovaries was normal and the adrenal glands showed a discrete enlargement. The long-acting GnRH analogue, triptorelin, was injected initially (3.75mg i.m.) and serum hormone levels were measured weekly throughout one month. RESULTS: GnRH produced a decrease in serum testosterone levels to normal values, in parallel with the suppression of serum LH and FSH concentrations. The patient was treated for three months with triptorelin and she experienced an amelioration of the hyperandrogenic symptoms. In order to achieve a diagnosis, the patient was submitted to a laparotomy that revealed a small hilus cell tumour in the left ovary. CONCLUSION: GnRH analogues may offer a good therapeutic option in some states of gonadotrophin-dependent hyperandrogenism of ovarian origin.
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3/16. Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma.

    Although nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, adenomatous transformation resulting in glucocorticoid excess is a rare phenomenon. We report a case of a 63-yr-old woman that presented with new-onset hirsutism, facial plethora, hypertension, centripetal obesity, and a proximal myopathy. The 24-h urinary free cortisol excretion rate was elevated, and the serum ACTH level was suppressed. The patient failed an overnight and low dose dexamethasone suppression test and did not respond to CRH stimulation. In light of the undetectable baseline morning ACTH levels and the blunt response to CRH, the diagnosis of corticotropin-independent Cushing's syndrome was made. Imaging studies revealed normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Dramatic resolution of symptoms was observed after surgical removal of the 3.5-cm mass. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. The case reported here represents the unusual circumstance in which the development of adenomatous transformation of ectopic adrenal tissue has been prospectively observed with imaging studies. It illustrates the importance of considering ectopic corticosteroid-secreting tumors in the context of corticotropin-independent Cushing's syndrome.
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4/16. testosterone-secreting gonadotropin-responsive adrenal adenoma and its treatment with the antiandrogen flutamide.

    A 55-year-old woman with virilization had an appreciably elevated testosterone level, which was not suppressed by dexamethasone, but was increased by stimulation with human chorionic gonadotropin (hCG). ultrasonography and computed tomography revealed an adenoma 2.5-3.0 cm in diameter in the right adrenal gland. The patient was treated with the antiandrogen flutamide in a daily dose of 500 mg for 4 months. A substantial regression of her hirsutism was observed during flutamide administration, but the serum testosterone level remained high. Right adrenalectomy was performed. Histologically, the tumor proved to be an adrenocortical adenoma of zona reticularis type. The adenoma tissue contained specific hCG receptors (187 fmol/g). The steroid concentration in the tumor tissue was examined by means of high pressure liquid chromatography-radioimmunoassay (HPLC-RIA). A significantly increased testosterone content was detected, and the levels of its precursors, androstenedione and dehydroepiandrosterone, were also elevated. Following adrenalectomy, serum testosterone concentration decreased to the normal level. The mechanism of the inappropriate regulation in the testosterone production of the adrenal tumor has not been fully elucidated.
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5/16. Androgen-secreting adrenal adenomas.

    BACKGROUND: The androgen source in women with hirsutism and signs of virilism may be the ovary or adrenal gland. CASES: Three patients with androgen excess are reported. Two had hyperandrogenemia and cushing syndrome with an adrenal mass greater than 5.5 cm; the third had a small adrenal adenoma secreting only testosterone and responsive to human chorionic gonadotropin. In all cases, the pathologic report from surgery and the long-term resolution of symptoms confirmed the benign nature of the tumors. CONCLUSION: Basal and dynamic hormonal tests cannot precisely differentiate ovarian from adrenal tumors. Adrenal adenomas must be considered as a cause of hyperandrogenic syndrome.
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6/16. Case report: acromegaly and Cushing's disease in a patient with synchronous pituitary adenomas.

    A 40-year-old white woman presented with hirsutism, amenorrhea, generalized fatigue, diffuse weight gain, acral changes, and coarsened facial features. physical examination revealed mild diastolic hypertension, acromegalic features, hirsutism, and seborrhea. The growth hormone concentration was elevated and did not suppress after glucose administration. Urinary free cortisol excretion was increased and was not suppressed during a 2 mg low-dose dexamethasone suppression test. magnetic resonance imaging of the sella demonstrated a 1.3 x 1.2 x 0.8 cm pituitary adenoma. Trans-sphenoidal resection was performed, and portions of the resected tumor were analyzed by routine pathologic methods. Histopathologic and immunohistochemical findings indicated discrete growth hormone- and adrenocorticotropic hormone-producing pituitary adenomas. Coexisting acromegaly and Cushing's syndrome due to pituitary neoplasia was previously reported in two patients. However, to the authors' knowledge, this represents the first description of a patient with acromegaly and Cushing's disease resulting from discrete synchronous adenomas of the pituitary gland as defined by modern histopathologic techniques.
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7/16. 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.
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8/16. Cutaneous manifestations in a patient with a long-term history of untreated ACTH-dependent Cushing's syndrome.

    Cushing's syndrome is accompanied by many different skin symptoms. A case of a 43-year-old female patient with unrecognized adrenocorticotropic hormone (ACTH)-dependent cushing syndrome is reported. Besides numerous skin lesions, the patient presented with a rapidly progressive leg ulcer due to venous thrombosis. The initial skin symptoms appeared on her lower leg one month prior to admission. The patient was treated with oral prednisone, however, rapid progression of the skin lesion was observed. On admission the patient presented with a large, very painful ulceration, partially covered with a crust, on the right lower leg with several satellite smaller ulcerations. The other leg showed no changes. Moreover, the patient had pronounced hirsutism and skin darkening on the face, and Muehrcke's lines were observed on all her nails. Microscopic findings of skin biopsy were uncharacteristic, with some signs of angiopathy and vasculitis. Based on the elevated serum levels of ACTH and cortisol, and the result of dexamethasone suppression test, ACTH-dependent cushing syndrome was diagnosed. Sonography of leg veins revealed a recanalized thrombus of the right popliteal vein with reflux. The patient was transferred to surgical department where both suprarenal glands were removed. Upon suprarenal gland removal, significant improvement of the patient's general condition and rapid healing of the leg ulcer were observed. Briefly, this patient is presented to point out that a leg ulcer caused by venous insufficency may mimic fulminant purpura or some other type of vasculitis in patients with cushing syndrome.
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9/16. Increased 3-hydroxy-3-methyl-glutaryl coenzyme a reductase activity in a virilizing adrenal carcinoma.

    HMG-CoA reductase activity was determined on microsomal preparations of an adrenal carcinoma and on a control adrenal obtained from palliative surgery for breast carcinoma. In both tissues we also measured [14C]pyruvate incorporation to study the formation of sterols. The endogenous adrenal content of cholesterol and its esters was quantitated. The content of various steroids was also determined in tissues and media before and after incubations in Krebs-Ringer. The carcinoma had a HMG-CoA reductase activity of 972.0 pmol/mg protein/min vs 13.8 for the control adrenal. The tumor incorporated 4.6 pmol of [14C]pyruvate per mg protein per 90 min into digitonin precipitable sterols compared to 0.5 pmol found for the control gland. Free cholesterol and cholesterol esters in tumoral tissue were 0.09/100 mg and 0.02/100 mg tissue respectively, compared to 0.18 and 2.56 in control tissue. The output of corticosteroids and androgens was very high when calculated for the whole tumor. These results suggest that the carcinoma had acquired a high capacity for de novo synthesis of cholesterol which could have served as substrate for the observed high plasma androgen level.
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10/16. Benign nonfunctional tumors of adrenal gland.

    Seven cases of benign nonfunctional adrenal tumors are presented. All except one were found accidentally by computerized tomography (CT) scan during a workup of an abdominal condition. In 1 patient the CT scan was ordered to evaluate hirsutism, but subsequent endocrine studies were found to be normal. Asymptomatic adrenal tumors found by abdominal CT scans are usually benign. Careful selection of individual patients and a possible nonsurgical follow-up are suggested.
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