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

In a 20 year period, from 1971 through 1991, 105 chromaffin tumours--excluding cervical ones--were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas--or ectopic pheochromocytomas--as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromocytoma, either synchronous (2) or metachronous (1). 7 cases occurred in a Von Hippel-Lindau syndrome (3 bilateral) and 4 in a neurofibromatosis setting (1 bilateral). 3 other cases were familial without evidence of MEN (including a case of triple tumour: bilateral and ectopic and another ectopic case). 2 other cases were associated with seemingly sporadic hyperparathyroidism. As a whole, in 34 of 75 benign pheochromocytomas or paragangliomas, the tumour was not intra-adrenal, unilateral and sporadic. Among those 75 tumours, 22 were not overtly hypertensive, including 10 out of the 41 seemingly intra-adrenal, solitary and sporadic. The pheochromocytoma, benign, intra-adrenal sporadic, hypertensive accounts for no more than 30% of the subphrenic catecholamine-secretin chromaffin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ? Reflections on a series of 105 surgically treated subdiaphragmatic chromaffin tumors]. 134 86

In a 21-year-old Caucasian women with von Hippel-Lindau disease, norepinephrine-producing adrenal pheochromocytoma was identified as the underlying cause of severe hypertension. She was found to have extremely elevated levels of circulating renin and aldosterone, and she was markedly hypokalemic. Administration of captopril further enhanced renin secretion, while her blood pressure improved. The patient became normokalemic following tumor removal, and her blood pressure decreased to normal levels with reestablishment of normal circadian blood pressure rhythm. This case demonstrates that, in the absence of renovascular or malignant hypertension, pheochromocytoma can be the underlying cause for the clinical syndrome of hypertension associated with severe hypokalemia and hyperreninemic hyperaldosteronism.
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PMID:Hyperreninemia and secondary hyperaldosteronism in a patient with pheochromocytoma and von Hippel-Lindau disease. 143 50

Tumors of the female genital tract may be associated with a variety of unusual clinical manifestations. Uncommon endocrine and paraendocrine syndromes include production of human chorionic gonadotropin by tumors other than those of germ cell origin, hyperthyroidism associated with struma ovarii and gestational trophoblastic disease, the carcinoid syndrome, the Zollinger-Ellison syndrome, hypercalcemia, Cushing's syndrome, hypoglycemia, hypertension related to renin or aldosterone production, hyperprolactinemia, inappropriate secretion of antidiuretic hormone, and virilization associated with Nelson's syndrome and placental site trophoblastic tumor. Paraneoplastic syndromes associated with gynecological tumors include disorders of the nervous system, connective tissue, and skin, as well as hematologic abnormalities and the nephrotic syndrome. Heritable and other congenital syndromes associated with these tumors are the Peutz-Jeghers syndrome, the nevoid basal-cell carcinoma syndrome, Ollier's disease and Maffucci's syndrome, hereditary leiomyomatosis, ataxia-telangiectasia, von Hippel-Lindau's disease, thyroid abnormalities associated with Sertoli-Leydig cell tumors, and Carney's complex. Other syndromes associated with tumors of the female genital tract include Meigs' syndrome, hyperamylasemia, uveal melanocytic lesions, and pyrexia.
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PMID:Clinical syndromes associated with tumors of the female genital tract. 175 57

During the past 20 years (1970-90), we had 24 patients with pheochromocytoma: 19 diagnosed clinically and 5 post-mortem. Their ages ranged from 17 to 74 (mean, 43.2 years). Males (n = 14) outnumbered females (n = 10), a 1.41:1 M:F ratio. A majority were symptomatic (95%), with a typical triad of headaches, palpitations and diaphoresis. Most frequent finding was hypertension (95%). It was sustained in 60% and paroxysmal in 35%. In 6 patients (25%) pheochromocytomas were bilateral, all familial. Fifteen were solitary adrenal tumors (63%); 3 (12.5%) were extra-adrenal: 2 intra-abdominal, and 1 cardiac paraganglioma of right atrium. Of 6 familial cases, 4 were associated to Von Hippel-Lindau (VHL) disease, while 2 were multiple endocrine neoplasia (MEN-II) patients. All familial cases were bilateral and in the adrenals. There were no malignancies. Among the 19 clinical cases pre-operative Dx was made by positive urine VMA or catecholamines urine levels: (95 and 100% sensitivity respectively). Preoperative visualization by CT or MRI was done in 62% of the most recent patients. In 5 earlier cases the diagnosis was made post mortem: 3 died of cerebral hemorrhage, 1 with a pons infarct and 1 with congestive heart failure (CHF). There were 2 post-operative deaths and another died 13 years later from thyroid medullary carcinoma. Of the 19 operated, 13 (68%) were cured. Thus pheochromocytomas retain considerable morbidity and some mortality. These rare tumors constitute a clinical diagnostic challenge yet a rewarding therapeutic experience for the alert physician.
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PMID:Pheochromocytoma: a twenty year experience at the University Hospital. 177 16

A patient with Von Hippel disease is described. The Von Hippel syndrome is a genetic disease characterized by a retinal angiomatosis and multiple tumors (renal and pancreatic sites are common). The pheochromocytoma is-on the contrary-very uncommon and the association with 2 pheochromocytomas has probably never been reported. In our case, a double pheochromocytoma (the first in 1973, the second in 1986) was present, in conjunction with a thyroid tumor and with an anomalous renal vein (circumaortic ring). The clinical picture was characterized by a severe hypertension and a fourth stage retinopathy. A correct diagnosis was established only 10 years after the clinical exordium. The thyroidectomy and the ablation of both the pheochromocytomas were necessary.
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PMID:[Von Hippel syndrome with recurrent pheochromocytoma]. 224 26

Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 6 years (1980 to 1986). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 6 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences, there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension.
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PMID:Recurrent pheochromocytomas in children. 226 59

Eight cases of pheochromocytoma in children were managed between 1958 and 1978. Headache, visual blurring, sweating, and hypertension were the most common findings. One patient presented with a hypertensive crisis during appendectomy. Three other children had a family history of pheochromocytoma and Hippel-Landau disease, thyroid carcinoma, and renal stones. The diagnostic approach was based on a high suspicion of the disease from the clinical picture. The most reliable laboratory investigations were urinary VMA, metanephrine, and catecholamines. I.V.P. and angiography were successful in localizing the tumor. In 4 cases, CT scan localized the tumor, although a second tumor was not seen in 1 case. Preoperative control of hypertension was achieved with phenoxybenzamine, propanolol, apresoline and reserpine in seven cases. The main anesthetic drugs used were pentothal, methoxyflurane, nitrous oxide, Innovar, and pancuronium. Tumors were located in the right adrenal in four, the left adrenal in two, both adrenals in one, and bilateral para-aortic sites in one. In two cases, hypertensive episodes during manipulation of the tumor were controlled with phentolamine. Ligation of the venous drainage from a tumor was associated with a sudden sustained fall in systemic blood pressure in six cases. In two, blood pressure remained elevated until a second tumor was found and removed. Hypotension was then corrected by the rapid infusion of fluid and blood, and vasopressors were not used. There were no postoperative complications and all children remained normotensive during the follow-up. The tumors of seven of the patients were benign. One tumor showed a low grade malignancy.
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PMID:Pheochromocytoma in children--an update. 741 63

This report describes the anaesthetic management of a women with a term gestation, Von Hippel Lindau disease (VHLD), and a phaeochromocytoma, scheduled for a combined phaeochromocytoma resection and Caesarean section. Von Hippel Lindau disease is characterized by diffuse haemangioblastomas of the central nervous system (CNS) and viscera. It is also associated with phaeochromocytomas and renal cell carcinomas. Patients frequently have asymptomatic spinal cord and intracranial pathology. The patient and her fetus presented a challenge because of the anaesthetic restrictions imposed by VHLD, and her pregnancy. She was also at risk of developing malignant hypertension from the phaeochromocytoma. The patient was not a candidate for regional anaesthesia because of the possibility of spinal cord haemangioblastomas. She had received adrenergic blockade with phentolamine (total 30 mg a day) and propranolol (total 40 mg a day) since the 27th wk of gestation in order to control hypertension secondary to the phaeochromocytoma. General anaesthesia was administered with aggressive management of hypertension with adrenergic blockers (labetalol 1.0 mg.kg-1 and esmolol 0.75 mg.kg-1) and sodium nitroprusside 1.5 micrograms.kg-1 (total). Before delivery of the baby, opioids, which could have resulted in a fetus with CNS depression, were avoided. After delivery, opioids (sufentanil 0.4 microgram.kg-1 x hr-1) were used to limit the use of inhalational anaesthesia which may contribute to uterine atony. Postoperative pain was managed with an intravenous narcotic infusion. Both patients had uneventful postoperative courses.
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PMID:Caesarean section and phaeochromocytoma resection in a patient with Von Hippel Lindau disease. 840 82

A number of medical conditions have been linked with renal-cell cancer, although the evidence is not consistent in every case. In a large international case-control study of renal-cell cancer, we examined, among other hypotheses, associations with a personal history of certain medical conditions and a family history of cancer of the kidney or thyroid. Relative risks (RR), adjusted for the effects of age, gender, body-mass index, tobacco smoking and study centre, were significantly increased by a history of kidney stones or thyroid or kidney disease. The RR were not altered by additional adjustment for hypertension, or when diagnoses were restricted to those made at least 5 or 10 years before 1987 (the usual "cut-off" date). The link with kidney injury is particularly likely to be affected by recall bias. Increased RR of borderline significance were found for kidney infection (RR, 1.2) and diabetes (RR, 1.4). Having one first-degree relative with kidney cancer was associated with a significantly increased risk of renal-cell cancer (RR, 1.6; 95% Cl, 1.1-2.4). Seven cases reported 2 first-degree relatives with kidney cancer. No controls had first-degree relatives with kidney cancer. None of our participants reported having von Hippel-Lindau disease. The data suggests that a few conditions of the kidney are strongly associated with renal-cell cancer and that heredity plays a role in a small proportion of cases.
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PMID:International renal-cell-cancer study. VI. the role of medical and family history. 864 39

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


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