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

A family was investigated with an increased occurrence of pheochromocytoma. Three males out of 7 family members showed biochemical and morphological evidence of catecholamine-secreting tumor. Plasma concentrations of norepinephrine, aldosterone, arginine vasopressin and renin activity, as well as urinary excretion of norepinephrine, were enhanced in all the 3 patients. The plasma level of atrial natriuretic peptide decreased. After surgical removal of the tumor the hormonal alterations tended to be normalized; however, plasma concentration of norepinephrine remained above normal. The elevated plasma norepinephrine was postoperatively accompanied by slightly increased urinary norepinephrine excretion in 1 patient without any detectable tumor. The severity of the disease was most pronounced in the youngest patient, while in the oldest one a 'silent' pheochromocytoma was found. The occurrence of familial pheochromocytoma in successive generations may suggest an autosomal-dominant inheritance.
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PMID:Vasoactive hormones in familial pheochromocytoma with predominant norepinephrine secretion. 307 6

Primary aldosteronism, Cushing's syndrome and pheochromocytoma are the most frequent endocrine hypertensive disorders. Following biochemical confirmation of the clinical diagnosis, mainly non-invasive imaging techniques are necessary to determine the cause of the hormone excess. Diagnosis of primary aldosteronism is confirmed by analysis of aldosterone and renin in peripheral venous blood. Differentiation between adenoma and idiopathic adrenal hyperplasia usually is achieved by computed tomography and isotope scan. A reliable test for the biochemical confirmation of Cushing's syndrome is the determination of urine-free cortisol. In patients with equivocal results the dexamethasone suppression test is performed. Differential diagnosis between ACTH-dependent Cushing's syndrome (pituitary or ectopic) and primary adrenal disorders can be made by determination of plasma-ACTH and -cortisol. Non-invasive localization procedures include computed tomography of the abdomen, computed tomography or magnetic resonance imaging of the pituitary gland, sonography and isotope scan. Diagnosis of pheochromocytoma is based on elevated catecholamine levels in urine or plasma. The tumor is localized by ultrasound, computed tomography or isotope scan.
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PMID:[Rational diagnosis of endocrine forms of hypertension]. 307 51

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

A 34-yr-old woman with hypertension (142/102 mmHg), hypokalemia, high plasma and urinary aldosterone and low plasma renin activity was studied. A left adrenal tumor and enlarged right adrenal gland were demonstrated by adrenal venography. During administration of dexamethasone (2 mg daily, for 3 weeks), urinary aldosterone excretion decreased abruptly from 22.5 to 9-11 micrograms/day, serum potassium increased and blood pressure fell to 120-130/80-90 mmHg. After left adrenalectomy, all manifestations improved with no medication. The resected adrenal gland revealed clear cell adenoma and micronodular adrenocortical hyperplasia. The patient was considered to be a rare case of glucocorticoid-suppressible hyperaldosteronism with an aldosterone-producing adenoma.
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PMID:A case of glucocorticoid-suppressible hyperaldosteronism with aldosterone producing adenoma. 320 6

Primary aldosteronism-characterized by hypertension, hypokalemia and metabolic alkalosis--is caused by benign adenomata or bilateral adrenal hyperplasia in most cases. Aldosterone producing carcinomata of the adrenal cortex are very rare tumors. As the histological classification is difficult, the diagnosis is often drawn from tumor size, very high levels of plasma aldosterone, severe hypokalemia and malignant behaviour. The prognosis is very poor: Overall median and 5 year survival rate from diagnosis of adrenocortical carcinoma are 14 months and 24%.
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PMID:[Malignant aldosteronoma in the differential diagnosis of Conn syndrome]. 321 80

Primary aldosteronism due to an adrenocortical adenoma is commonly known as surgically correctable hypertension. Forty-three cases of primary aldosteronism were treated operatively at our Department between 1960 and 1985. Forty of them were operatively, found to have aldosteronoma with a mean size of 1.88 cm and weight of 2.67 g. The patients average age was 39.4 years old and the male to female ratio was 1 to 3. Adrenal phlebography, selective adrenal venous sampling, adrenal scintigraphy, CT and echography were used to preoperatively locate the tumor, and their diagnostic value was 83, 78, 76, 87 and 60%, respectively. Unilateral adrenalectomy or unilateral partial adrenalectomy was performed extraperitoneally with lumbar oblique incision in 32 of the 43 operated patients. Since the application of the preoperative diagnosing studies above mentioned, tumor localization has been clearly demonstrated in about 90% of the patients and the unilateral extraperitoneal approach is proved as a safe operative method with minimal blood loss of less than 200 ml and performed within two hours. Unilateral adrenalectomy by the flank approach has now become the operation of choice for the treatment of an aldosterone-producing adrenal adenoma.
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PMID:[Surgical management of primary aldosteronism--progress in localization studies and operative treatment]. 330 70

A 23-year-old white male was referred for hypertension resistant to triple antihypertensive treatment, with hypokalemia, hyperaldosteronism and elevated levels of circulating plasma renin activity (PRA). Renal angiography and echoscans put in evidence an avascular solid mass at the midlower level of the right kidney. Renal vein catheterization with sampling of blood from the lower branches of the right renal vein showed lateralization of renin secretion from that side. After surgical exeresis, the mass (1.0 cm) was diagnosed as a renal hemangiopericytoma on the basis of light and electron microscopy. Tumor exeresis was followed by a prompt normalization of blood pressure and plasma potassium, with a decrease in PRA and aldosterone. Two months after surgery the patient was still normotensive. Circulating levels of inactive (trypsin-activable) renin were around 60% of the total pool of plasma renin, i.e. much lower than those reported in other cases of renin-secreting tumors. After surgery, inactive and active renin fell in parallel, implying that both were secreted by the tumor. Tumoral PRA responded to postural stimulation, but was unresponsive to acute converting enzyme inhibition, suggesting that sympathetic stimuli were still operative, but the negative feedback inhibition by angiotensin II on renin secretion was lost. Acute converting enzyme inhibition by captopril dropped blood pressure; however, during long-term treatment, the drug (3 X 50 mg/day) was ineffective in terms of either blood pressure normalization or relief of secondary hyperaldosteronism. Acute calcium entry blockade by nifedipine (10 mg p.o.) caused an evident blood pressure drop.
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PMID:A renin-secreting tumor. 330 96

Five cases of primary aldosteronism are reported, and several techniques to localize the tumor were evaluated. On computed tomography which is a noninvasive technique, low density areas were identified in all cases, and definite adenomas were clearly detected in three of five cases. Adrenal scintigraphy with 131I-19-iodocholesterol is another noninvasive technique that showed unilaterally increased uptake in two cases. Dexamethasone inhibits the uptake of labeled cholesterol by hyperplastic or normal glands but does not interfere with the uptake by an adenoma. Using this technique, it was possible to lateralize additionally the tumor in one case. Adrenal venography which is an invasive technique that showed a circular deformity with tumor depicted the tumor in only one case. However, the venous effluent was sampled simultaneously from both glands and the plasma aldosterone concentration in each sample assayed. An increase in aldosterone concentration on only one side localizes the aldosterone producing adenoma to that side. The method was highly accurate, and provided the correct diagnosis preoperatively in all cases. Being a noninvasive technique, computed tomography and adrenal scintigraphy should be undertaken initially, and venography coupled with measurements of aldosterone concentrations in the left and right adrenal venous effluent could be a definitive procedure before surgery.
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PMID:[Five cases of primary aldosteronism: a comparative study on diagnosis for localization of adrenal tumor]. 340 May 42

A 27-year-old woman with an adrenal tumor that produced renin and aldosterone, associated with hypertension and adrenogenital syndrome, is described. Severe hypertension, cardiomegaly, a low serum potassium level, clinical symptoms of adrenogenital syndrome, and a left upper abdominal tumor also were found. Endocrinological studies showed that plasma and urinary levels of sex steroid hormones such as dehydroepiandrosterone, androsterone, and testosterone were markedly increased. Plasma renin activity, plasma angiotensin II, and plasma aldosterone levels also were increased markedly, although deoxycorticosterone levels remained within the normal range. The possibility of renovascular hypertension was excluded by angiography of the renal artery and by venous sampling of plasma renin activity. Abnormal elevations in plasma aldosterone levels persisted despite normalization of plasma angiotensin II by converting enzyme inhibitor administration. It was suspected that this patient had an adrenal tumor producing renin as well as sex steroids and aldosterone. Microscopy of the resected tumor revealed that the tumor was composed mostly of cells with large nuclei and light cytoplasm. The tumor contained dehydroepiandrosterone, dehydroepiandrosterone sulfate, testosterone, aldosterone, and renin. Immunohistochemical study showed that some of the tumor cells produced renin. Biopsy of the left renal tissue showed evident atrophy of the juxtaglomerular cells and pronounced arteriosclerosis. After resection of the tumor, all blood and urinary levels of the abnormally increased hormones returned to a normal range and an apparent fall of blood pressure was noted. To our knowledge, this is the first report of a renin and aldosterone-producing adrenal tumor associated with hypertension and adrenogenital syndrome.
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PMID:A case of adrenal tumor producing renin, aldosterone, and sex steroid hormones. 353 Oct 7

The most important functional tumors of the adrenal cortex are those that secrete cortisol or aldosterone in excess. Biochemical testing when appropriately utilized can diagnose and differentiate the cause of Cushing's syndrome, and when an adrenal adenoma is found, surgical excision is curative. The diagnosis and surgical treatment of primary aldosteronism are straightforward today, and localization of the usual small cortical tumor producing the syndrome can be achieved by CT and NP-59 scanning or selective venous assays. Adrenocortical carcinomas are relatively rare, are usually incurable when diagnosed, and are an important consideration in the incidentally discovered adrenal mass found by CT scanning.
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PMID:Diagnosis and treatment of functioning and nonfunctioning adrenocortical neoplasms including incidentalomas. 355 Nov 52


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