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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary aldosteronism is the commonest cause of potentially curable hypertension when diagnosed in both florid and less florid forms. Genetic screening, so far available only for glucocorticoid-suppressible hyperaldosteronism, permits diagnosis from birth, before any biochemical or clinical abnormalities appear. Biochemical screening using the aldosterone-to-renin ratio permits diagnosis in the absence of raised aldosterone or of hypokalemia. Primary aldosteronism occurs in several familial forms. As well as the variety described in 1966 which is ACTH-dependent and glucocorticoid-suppressible, and not so far associated with tumors, another variety described in 1991 is not glucocorticoid-suppressible and is frequently associated with aldosterone-producing adenomas (APAs). Primary aldosteronism due to adrenocortical hyperplasia, adenoma, or carcinoma can also occur as part of the multiple endocrine neoplasia syndromes, where normoplasia, hyperplasia, benign neoplasia, and malignant neoplasia can exist in the same patient in the same endocrine gland(s) at the same time. The morphology of adrenocortical hyperplasia causing primary aldosteronism ranges from glomerulosa-like (idiopathic hyperplasia of the adrenals) to fasciculata-like (glucocorticoid-suppressible hyperaldosteronism). The morphology of adrenocortical neoplasia causing primary aldosteronism can also be either predominantly glomerulosa-like or fasciculata-like, in our experience equally often. Varying morphology of APAs is associated with varying responses of aldosterone to angiotensin II. Tumors predominantly fasciculata-like are unresponsive to angiotensin II, whereas those predominantly glomerulosa-like are responsive to angiotensin II. Both subtypes can be seen in a single family. Primary aldosteronism represents a spectrum of genetic disorders resulting in hyperplasia or neoplasia, but all are associated with some degree of autonomy of aldosterone production, independent of the renin-angiotensin system.
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PMID:Primary aldosteronism--some genetic, morphological, and biochemical aspects of subtypes. 779 13

Pheochromocytoma, although rare, is associated with a high degree of morbidity and mortality if not recognized. A high degree of suspicion in patients with new-onset hypertension; hypertension with sudden worsening or development of diabetes mellitus; or a family history of MEN, neuroectodermal tumors, or simple pheochromocytoma should prompt biochemical confirmation with either 24-hour urine catecholamines (norepinephrine and epinephrine) or total MET (NMET plus MET). Following confirmation of the diagnosis, radiologic studies with CT and (if needed) MIBG are employed to localize the tumor. Surgical removal is the only definitive therapy. Medical management with alpha-blocking agents, to control symptoms and prevent a hypertensive crisis, is generally advocated for 2 weeks preoperatively and intraoperatively. Occasionally, beta-blockers, employed only after adequate alpha-blockade, are necessary to control tachycardia and tachyarrhythmias. High-dose MIBG and combination chemotherapy have been used adjunctively to treat malignant pheochromocytoma, although neither modality provides lasting satisfactory results. Normal urine assays performed 2 weeks postoperatively ensure the complete removal of all tumor. Additionally, lifelong follow-up (yearly initially) is necessary to detect any signs of benign recurrence or malignancy because these have been reported to occur as long as 41 years after the initial surgical resection. Biochemical evidence of excess catecholamine production usually precedes the clinical manifestations of catecholamine excess when these tumors recur.
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PMID:Pheochromocytoma. Update on diagnosis, localization, and management. 780 88

To clarify the role of a novel vasoactive peptide endothelin in endocrine hypertension, we examined endothelin (ET)-like immunoreactivity and the ET binding sites in the tissues of two pheochromocytoma. Case 1 was a 25-year-old woman who manifested multiple endocrine neoplasia (MEN) II b with left adrenal pheochromocytoma, and case 2 was a 48-year-old woman with familial bilateral pheochromocytoma. In both cases, fairly high amount of ET-like immunoreactivity was detected in the resected tumors. By competitive binding analysis, high affinity binding sites for 125I-ET-1 were also detected in the same resected tissues. Concomitant existence of ET and its receptor suggests that ET may play an important role in these tumors in modulating the secretion of catecholamines from the tumors by an autocrine/paracrine fashion.
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PMID:[The role of endothelin in adrenal medulla: identification of endothelin and its receptor in two cases of pheochromocytoma]. 809 82

A 49-year-old woman was diagnosed in 1985 as having pheochromocytoma because of hypertension with high levels of plasma catecholamine concentration and 24-hour urine excretion of vanillyl-mandelic acid and metanephrine together with a right adrenal mass. The excised tumor cells had fine granular basophilic cytoplasm with argyrophilic granules by Grimelius' method. Four years later, she was diagnosed as having a duodenal bulb ulcer. Serum gastrin showed an abnormally high level of 1900 pg/ml. Abdominal echogram and computed tomography revealed a hypoechoic lesion in the pancreas and intrahepatic multiple tumors. A needle biopsy specimen of the liver tumor was compatible with the histology of metastatic islet cell tumor. A diagnosis of Zollinger-Ellison syndrome was made due to malignant gastrinoma with multiple liver metastases. The patient had no family history of endocrinological or neoplastic disorders. The present case indicates the possibility that pheochromocytoma and gastrinoma, that is, endocrine tumors characteristic of multiple endocrine neoplasia (MEN) I and MEN II, may be coincident even in a person without MEN. A continued awareness of previously rare or undescribed manifestations is important in patients with islet cell tumors or pheochromocytoma.
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PMID:Zollinger-Ellison syndrome and pheochromocytoma. Report of a case. 809 84

The unusual case of a 65-year-old woman with intermittent hypotension, fever, pulmonary edema and coma as initial presentation of pheochromocytoma is reported. The patient developed respiratory, cardiac and renal failure, disseminated intravascular coagulation and liver dysfunction. She had to be defibrillated on multiple occasions, occurring in periods of severe hypertension. After successful surgical removal of a pheochromocytoma a thyroid medullary carcinoma was detected. Several members of the patients family had presented with multiple endocrine neoplasia (MEN II).
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PMID:Multiple organ failure and coma as initial presentation of pheochromocytoma in a patient with multiple endocrine neoplasia (MEN) type II A. 810 32

Expansive lesions of the adrenal glands without signs of hormonal hypersecretion are usually discovered "incidentally", and are therefore called "incidentalomas". Since the silent adrenal masses constitute a heterogeneous group of lesions, the most important issue raised by the identification of these masses is their possible malignant potential. Therefore, the age and sex of the patients, the size of the mass, its imaging characteristic and its histologic features are the most important factors in the assessment of nonfunctioning adrenal masses. We report here the case of a women, aged 43 years mild hypertension, harbouring a great adrenal mass of 8 cm diameter, with cystic appearance at CT scan and ultrasonography, it did not show any uptake after 75Se-Seleniumcholesterol or 131I-Metaiodobenzyl-guanidine. The patient was also affected by mild hyperprolactinemia caused a small pituitary adenoma. After selective venography, she underwent the surgical removal of the left adrenal gland with its mass, which showed a hystological picture of adrenal adenoma. The postoperative course was clinically normal. The interest of this case resides: in the dimensions of the adrenal tumor (the presence of adenomas greater than 6 cm diameter is extremely rare); in the radiographic findings, showing a cystic appearance, probably related to a fluid component, in the association of adrenal and pituitary adenoma, as a possible variant of multiple endocrine neoplasia (MEN) of type I.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of adrenal adenoma with radiologic "cystic" appearance associated with hypophyseal adenoma]. 815 66

Hypertension attributable to pheochromocytoma is a very attractive model for the elucidation of the pathogenesis of hypertension. Sixteen different point mutations in the RET proto-oncogene and 30 mutations in the Von Hippel-Lindau (VHL) tumor suppressor gene have been identified so far associated with expression of pheochromocytoma. Each of these mutations initiates either the syndrome of multiple endocrine neoplasia type 2 (MEN 2) (MEN 2A and MEN 2B) or the VHL disease. Certain mutations in both genes are associated with the presence of pheochromocytoma. In general, these pheochromocytomas produce catecholamines that result in hypertension. Therefore, analysis for germline mutations in these genes are of practical value, because susceptibility to these diseases can be predicted in as yet clinically unaffected relatives.
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PMID:Monogenetic hypertension and pheochromocytoma. 880 29

Pheochromocytomas are catecholamine-producing tumors, representing one of the most important causes of secondary hypertension. The classification of these tumors considers both sporadic and familial forms, intra- and extraadrenal localization as well as the dignity. Familial pheochromocytomas are primarily seen under the conditions of multiple endocrine neoplasia, von Hippel-Lindau disease or neurofibromatosis type 1. The list of clinical symptoms includes hypertension, which can be both continuous or intermittent, headache, tachycardia and sweating. It is most important to standardize the pre-analytical procedures, i.e. control for sampling conditions and adequate choice of parameters in plasma or urine. For screening sensitive methods will be employed (free catecholamines in 24h-urine) and for confirmation of the diagnosis, specific procedures are performed (Clonidine test, MIBG-scintigraphy). The endocrinological and biochemical procedures are completed by molecular genetic techniques in familial pheochromocytoma.
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PMID:[Clinical and endocrine diagnosis of pheochromocytoma]. 933 11

We experienced a case of MEN type 2a with bilateral and large pheochromocytomas. A 39-year-old man was admitted to the previous hospital with complaints of paroxysmal headache. hypertension and diabetes mellitus. Radiographic imagings showed thyroid tumors in both lobes and bilateral adrenal tumors. Thyroid tumors were histologically proved to be medullary thyroid carcinoma by needle biopsy and systemic investigations revealed an excessive secretion of plasma and urinary cathecholamines which suggested the presence of pheochromocytoma. The patient was diagnosed as MEN type 2a. He was admitted to our hospital for the treatment of bilateral adrenal tumors for which we performed one-stage bilateral adrenalectomy by thoracoabdmonal approach. Both adrenal tumors were histologically confirmed as pheochromocytoma. The patient's postoperative course was uneventful. He underwent uneventful total thyroidectomy approximately 2 months after bilateral adrenalectomy. Even in bilateral and large pheochromocytomas, one-stage bilateral adrenarectomyenables safe postoperative managements. We concluded that the thoracoabdominal approach is feasible in the patients with huge and cranially spreading adrenal tumor, which gives us a wide operative field for easy vascular control.
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PMID:[One-stage-bilateral adrenalectomy by thoracoabdominal approach for bilateral large pheochromocytomas in men type IIa. A case report]. 938 71

A 5-cm pheochromocytoma located in segment 7 of the liver was found incidentally in a 45-year-old man with mild diabetes mellitus and hypertension, and resected. The tumor was demonstrated by computed tomography and magnetic resonance imaging to have completely invaginated itself into the liver and to be receiving blood from a dilated right hepatic artery alone. Surgery revealed the hepatic mass to be tightly adherent to the right adrenal gland. The histopathologic diagnosis was pheochromocytoma growing exophytically from the right adrenal gland. There was no association with multiple endocrine neoplasia type 1 and type 2. A postoperative 131I metaiodobenzylguanidine scan revealed no accumulation, and the patient is currently doing well without recurrence or hypertension one year after the operation. A pheochromocytoma deeply invaginating into the liver should be considered in the differential diagnosis of primary hypervascular hepatic tumors.
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PMID:Pheochromocytoma growing exophytically from the right adrenal gland and invaginating into the liver. 939 Feb 15


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