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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenocortical carcinoma (ACC) is a rare disorder with an estimated incidence of only 0.023 percent of all malignancies. In most cases, Cushing's syndrome and virilization or feminization due to abnormal steroid production by the tumor rapidly lead to the diagnosis. Occasionally, the tumor produces an excessive amount of mineralocorticoids only and ACC can be revealed by an isolated syndrome of primary aldosteronism. Out of 100 cases of tumoral primary aldosteronism studied from 1977 to 1987, we observed 4 ACC and 96 Conn's adenomas (CONN). When primary aldosteronism was diagnosed, ACC and CONN had same clinical features, although hypokalemia in ACC was more profound: 2.2 +/- 0.76 mmol/l (1.4 to 3.2) compared to 2.9 +/- 0.5 (1.6 to 4.2) in CONN. Mean supine plasma aldosterone levels, plasma renin and aldosterone responses to the upright posture or to serum saline infusion, cortisol at 8 a.m. were not different in patients with ACC from those observed in patients with CONN. 24 hours urinary cortisol excretion and 17-ketosteroids excretion were highly increased in three out four patients with ACC. Clinical, biological and hormonal investigations were therefore not sufficient to diagnose malignant tumoral primary aldosteronism. Systematic computed tomographic scanning allowed to differentiate carcinomas from adenomas on the following criteria: ACC showed enlarged tumor size that was always above 30 mm in diameter, whereas the largest CONN measured 20 mm.ACC appeared as an heterogeneous tumor with the presence of internal calcifications in each case of ACC, that were diagnosed both on ultrasound and CT scan, whereas none of the CONN showed any calcification, using the same screening procedure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Malignant adrenal cortex carcinoma revealed by an isolated picture of primary hyperaldosteronism]. 314 35

Eight cases of adrenocortical tumor are presented with a review of the literature. Although such tumors are rare, they are important causes of inappropriate virilization and Cushing's syndrome in childhood. Clinical virilization with or without hypercortisolism was found in all eight children, who were 5 years old or younger. Excessive linear growth was noted, despite evidence of hypercortisolism. Serum levels of dehydroepiandrosterone, dehydroepiandrosterone sulfate, testosterone, and cortisol were elevated in all cases tested and appear to be useful diagnostic alternatives to the more traditional determinations of urine 17-ketosteroids and 17-hydroxycorticosteroids. Abdominal sonography and computed tomography have proven to be reliable tools for tumor localization. Surgical resection was the definitive therapy in all patients, and perioperative steroid replacement was essential. Histologic diagnosis appeared to have little bearing on prognosis, and the majority of pediatric patients have had clinically benign disease. At a mean follow-up of 3 years, seven of the eight children were alive and had no evidence of tumor recurrence.
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PMID:Virilizing adrenocortical tumors in childhood: eight cases and a review of the literature. 316 51

A 73-yr-old woman had noted progressive virilization. The plasma testosterone levels were elevated (340 ng/dl) and the pelvic ultrasound examination revealed an enlarged left ovary. At laparotomy, a large cystic tumor replaced the left ovary. The plasma testosterone became normal after the removal of the tumor. On anatomical analysis, the tumor was an ovarian cystadenoma with hyperplasia of stromal cells in the periphery of the tumor.
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PMID:Ovarian cystadenoma with stromal cell hyperplasia and postmenopausal virilization: a case report. 316 60

A case of lipid (lipoid) cell tumor of the ovary without masculinization is reported. The age of the patient was 46 years.
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PMID:[Lipid- (lipoid-) cell tumor of the ovary]. 318 18

A 51-year-old woman presented with hirsutism and virilization of gradual onset. The serum gonadotropin concentrations were in the postmenopausal range, the serum testosterone concentration was markedly elevated (9.8 nmol/l) and the serum estradiol concentration (220 pmol/l) was elevated above the postmenopausal range. A selective venous catheterization study demonstrated raised serum testosterone and androstenedione levels in ovarian veins and suggested the presence of a left ovarian tumor. The raised peripheral estradiol level was shown to be due to ovarian hypersecretion. After bilateral oophorectomy the serum testosterone became normal. Ovarian histology revealed bilateral stromal hyperthecosis. Ovarian hyperthecosis is a rare but important cause of serum testosterone levels in the neoplastic range. This is the third case reported of postmenopausal virilization due to ovarian hyperthecosis and the first report of a selective venous catheterization study in such a patient.
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PMID:Virilization due to ovarian hyperthecosis in a postmenopausal woman. 323 54

A 15-year-old girl who presented with primary amenorrhea and virilization had an adrenocortical adenoma that secreted predominantly testosterone. To our knowledge, she is the first peripubertal and second youngest patient with a testosterone-secreting adrenal tumor described. Serum dehydroepiandrosterone sulfate and urinary 17-ketosteroid and 17-hydroxycorticosteroid levels were normal. A tumor was located by a computed tomographic (CT) scan and by uptake of 6-beta-[75Se] selenomethylnorcholesterol. Microscopic examination of the tumor showed typical features of an adrenocortical adenoma with no histologic features characteristic of Leydig cells. Postoperatively, her hirsutism regressed, she rapidly went through puberty, and regular monthly menstruation started four months later. Finding the source of testosterone in a virilized patient can be difficult. Eleven of the 14 previously described patients with testosterone-secreting adrenal tumors initially underwent misdirected surgery on the ovaries. Review of these cases revealed that results of hormone stimulation and suppression tests are unreliable and that these tumors are usually large. Therefore, CT scanning of the adrenal glands is recommended in all patients suspected of having a testosterone-secreting tumor. If the adrenal glands on CT scan are normal, then surgery directed at the ovaries can be undertaken. Adrenal and ovarian vein catheterization is rarely necessary.
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PMID:Testosterone-secreting adrenal adenoma in a peripubertal girl. 331 60

In a patient with Nelson's syndrome, elevated peripheral concentrations of androgens usually associated with neoplasm prompted ovarian vein catheterization. Androgen excess was limited to the right ovary. However, laparotomy revealed bilateral multiple paraovarian nodules with histologic appearance of adrenocortical tissue. The occurrence of virilization from ectopic adrenal tissue with markedly elevated ACTH concentrations is exceedingly rare.
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PMID:Virilizing paraovarian tumors: a consequence of Nelson's syndrome? 337 20

A 27-yr female patient was admitted to the hospital because of secondary amenorrhea and a severe and complex virilization syndrome. The urinary 17-Ks, 17 OHCS and testosterone glucuronide values were greatly increased. The plasma testosterone through very high, was within the normal limits in males (3.52 ng/ml). Urography, echography and CT scan revealed a large right adrenal tumor. Removal of the tumor brought about a considerable clinical improvement: reoccurrence of the menstrual cycle, a progressive decrease in the virilization syndrome and normalization of the urinary 17-Ks, 17-OHCS and of the plasma testosterone.
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PMID:Unusually large adrenal adenoma excreting unusually large amounts of androgen metabolites in urine. 338 88

Nine cases of adrenocortical tumor are presented, six were males. Four were less than three years and five were between 5 and 10 years of age. Clinical virilization was found in 8 children, one had only signs of hypercortisolism and another showed signs of virilization and hypercortisolism simultaneously. Urinary 17-KS and 17-OHCS were high in all patients. Plasma levels of testosterone and of the other adrenal androgens were high in all the cases tested. Plasma level of cortisol was elevated only in few cases. In two out of five cases steroids were only partially suppressed by dexamethasone. Computed tomography and abdominal sonography have been useful tools for the localization of the tumour. The resection of the tumour, independent of histopathological diagnosis, led to a complete normalization of the clinical and hormonal picture in eight cases evaluated at a distance of 2 months 10 years after surgery. In one case a hepatic metastasis was observed and removed three years after surgery.
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PMID:Adrenocortical tumours in children: our experience with nine cases. 346 66

We report here the case of a 22-month-old girl with virilization due to a potentially malignant adrenal tumor. She presented with clitoromegaly and growth of pubic hair, first noticed at birth. The clinical picture, hormonal profile, and pathologic findings are described. The practical aspects of the differential diagnosis and treatment are illustrated.
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PMID:Virilization of a female infant due to an adrenal tumor. 352 12


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