Cases reported "Feminization"

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1/10. Cosecretion of estrogen and inhibin B by a feminizing adrenocortical adenoma: impact on gonadotropin secretion.

    We describe the first reported case of a feminizing adrenocortical adenoma cosecreting estrogens and inhibin B. A 39-yr-old man, with no previous history of disease and free of treatment, complained of gynecomastia without any clinical abnormality. plasma E2 and T were 496 pmol/liter and 8.7 nmol/liter, respectively. Testicular echography was normal, and abdominal computed tomography scan showed a 28-mm right adrenal tumor. hCG (5000 IU, im) induced a rise in plasma T levels (20.7 nmol/liter) without any change in plasma E2 levels. Basal plasma LH and FSH levels were undetectable. GnRH (100 microg, i.v.) induced an increase in plasma LH levels without a change in plasma FSH levels. The mean plasma inhibin B level was 330 /- 45 pg/ml (normal range, 94-327). Pulsatile GnRH administration (20 microg/pulse every 90 min for 3 d) stimulated LH secretion, whereas FSH secretion remained blunted. The patient underwent surgery to remove a 12-g adrenal adenoma. Six months later, plasma E2 and T levels were normalized. LH showed a spontaneous pulsatile pattern, and the mean plasma FSH level was 4.8 U/liter. The secretion of both gonadotropins was stimulated during a pulsatile GnRH administration performed in the same manner as before surgery. The mean plasma inhibin B level was 210 /- 25 pg/ml. Immunohistochemical studies revealed the presence of aromatase in clusters of tumor cells. Incubation of tumor sections with anti-beta(B)-inhibin antibody revealed intense staining in groups of cells that were also labeled with anti-alpha-inhibin antibody. These data show that the tumor cosecreted E2 and inhibin B, which were both responsible for inhibition of gonadotropin secretion. Tumor secretions appeared to be much more potent in suppressing FSH than LH levels.
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2/10. Major hyperestrogenism in a feminizing adrenocortical adenoma despite a moderate overexpression of the aromatase enzyme.

    A 30-year-old male was referred for the rapid development of gynecomastia, and dramatic hyperestrogenemia was assessed: plasma estrone, estradiol but also cortisol were not suppressed by high-dose dexamethasone, while gonadotropin pulsatility was completely abolished. A 60-mm right adrenal tumor was evidenced on computed tomography-scan, and the patient underwent adrenalectomy. The tumor was found to express a moderate increase in aromatase activity compared with adjacent non-neoplastic adrenal tissue. Quantitative RT-PCR also showed a weak and non-significant increase in total aromatase mRNA in the tumor compared with normal adrenal tissue. aromatase transcripts were mainly promoter PII-derived, but different patterns of aromatase minor transcripts were found: promoter I.3- and I.6-derived transcripts were identified in the tumor, while only promoter I.4-derived transcripts were found in normal adrenal. This case report demonstrates that a sharp aromatase overexpression is not a prerequisite for clinical and biochemical hyperestrogenism, and further characterizes the aromatase promoter utilization in this feminizing adrenocortical tumor and in the normal adrenal cortex.
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3/10. Feminizing adrenocortical tumor. Histological and ultrastructural study.

    A case of a feminizing adrenocortical tumor associated with Cushing's syndrome in a 29 year old male is presented. The ultrastructural features are compared with adrenal tumors secreting aldosterone, glucocorticoids of androgens. As in adrenal carcinomas, this tumor demonstrates nuclear pleomorphism with enlarged nucleoli and nuclear pseudoinclusions. The cytoplasmic organelles show some parallels between feminizing and androgen-secreting adrenal tumors. Different types of mitochondria occur with varying amounts of smooth endoplasmic reticulum. Numerous microbodies are present. Histological and ultrastructural signs indicating probably malignancy are discussed and it is noted that most of the feminizing adrenal tumors are carcinomata. Neither local recurrence nor distant metastases have yet been detected in this case, two years after excision of the tumor.
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4/10. Feminizing adrenocortical tumors in male patients: adenoma versus carcinoma.

    We report a case of a feminizing adrenocortical adenoma in a 8-year-old boy and feminizing carcinoma in a 25-year-old man. Because diagnosis of adrenal malignancy in such tumors by histopathological criteria is not always conclusive, a clinicoradiological approach may be used in the evaluation.
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5/10. flow cytometry in feminizing adrenocortical carcinoma.

    A case of feminizing adrenal cortical neoplasm is presented in which automated flow cytometry determination of deoxyribonucleic acid content was used to confirm malignancy.
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6/10. Feminizing adrenocortical carcinoma with Cushing's syndrome and pseudohyperparathyroidism.

    A patient with adrenocortical carcinoma had three major endocrine abnormalities attributable directly to the tumor: hypercortisolism (Cushing's syndrome), hyperestrogenism (feminization), and hypercalcemia (pseudohyperparathyroidism). There were higher levels of immunoreactive parathyroid hormone in venous effluent from the tumor or its abdominal metastases compared to that found in the veins draining the parathyroid glands. This, together with the presence of normal parathyroid glands on autopsy, established the diagnosis of pseudohyperparathyroidism as the cause of hypercalcemia in this patient.
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7/10. Estrogen-producing adrenocortical carcinoma. A light and electron microscopic study.

    A case of adrenocortical carcinoma with feminization seen in a man aged 35, is reported. The levels of estron (E1) and estradiol (E2) in the venous blood draining the tumor were high, and it was confirmed by in vitro assay of tumor cells taken from the primary tumor of the left adrenal gland that the tumor produced estrone. The light microscopic examination demonstrated that the primary tumor was composed of mixture of large cells with pleomorphic nuclei and vacuolated cytoplasm and uniform cells with ovoid nuclei and eosinophilic cytoplasm. The electron microscopic examination on the latter cells revealed numerous large and irregularly shaped mitochondria with mostly tubular or lamellar and occasionally vesicular cristae and electron-dense matrix, well-developed smooth-surfaced endoplasmic reticulum in the cytoplasm. However, lipid droplets and lysosomes or lipofuscin granules were scanty. From these findings, it is suggested that cells of the present tumor have characteristics of those in the zona reticularis of the adrenal cortex as well as in the fetal cortex, and the functional property of this tumor is well correlated with its morphological features.
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8/10. Feminizing adrenocortical carcinoma in man.

    A 37-year-old male with adrenocortical tumor presenting marked gynecomastia and impotence as initial symptoms was experienced. Abnormal elevations in urinary 17-KS, 17-OHCS and blood estrogen levels were noted. Total surgical removal of the tumor weighing 800g was performed. Postoperatively the gynecomastia disappeared and he regained libido with no signs of recurrence one year later.
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9/10. feminization as a result of both peripheral conversion of androgens and direct estrogen production from an adrenocortical carcinoma.

    A 45-year-old man presented with gynecomastia, hypertension and a large left adrenal mass. Further evaluation revealed elevated serum concentrations of estrogen, estrone sulfate, androstenedione, dehydroepiandrosterone, dehydroepiandrosterone sulfate, deoxycorticosterone, and aldosterone and increased 24-hour urinary 17-ketosteroid and free cortisol excretion. Removal of a 10 kg adrenocortical carcinoma led to normalization of the hormone concentrations and partial resolution of the gynecomastia. There was no clinical evidence of metastases. Incubation of tumor slices demonstrated that the tumor had an active aromatase and sulfotransferase. We estimated that about half the serum estrone arose from peripheral conversion of androstenedione. Feminizing adrenocortical carcinomas are rare and this case is unusual given the lack of clinical metastases and the probable dual source of estrogen from tumor as well as from the peripheral conversion of tumor-derived androgens.
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10/10. A feminizing adrenocortical carcinoma presenting with gynaecomastia.

    A case is presented in which gynaecomastia was the sole initial presenting symptom of a feminizing adrenocortical carcinoma. This rare pathological lesion is discussed.
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