Cases reported "Pheochromocytoma"

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1/22. Severe paroxysmal hypertension (pseudopheochromocytoma): understanding the cause and treatment.

    Severe, symptomatic paroxysmal hypertension always generates suspicion of a pheochromocytoma, a catecholamine-secreting tumor. However, most patients with this disorder do not have this tumor and their condition remains undiagnosed and ineffectively treated. This case series, summarizing the course of 21 such patients, suggests a cause and an effective treatment approach. All 21 patients insisted that the paroxysms were not related to stress or emotional distress, initially discouraging consideration of a link to emotions. Nevertheless, with careful psychosocial interviewing, the disorder could be attributed to emotions patients were not aware of, and, therefore, unable to report. Such emotions were related either to previous severe emotional trauma or to a general tendency to keep distressful emotions out of awareness. With treatment based on this understanding, further paroxysms were eliminated in 13 (62%) of 21 patients. Alpha- plus beta-blockade was used, combined, when necessary, with an antidepressant agent, with or without an anxiolytic agent. In 3 cases, the disorder was cured with psychotherapy alone. Because the presenting symptoms are physical rather than emotional, patients present to internists and primary care physicians rather than to psychotherapists. For this reason, more awareness of this disorder in the medical community is needed.
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2/22. Difficult anesthetic management during pheochromocytoma surgery.

    The anesthetic management of two middle-aged patients having surgical removal of large pheochromocytomas is described. The same team of physicians was involved in the care of the patients, including an endocrinologist, who supervised their preoperative care. Although the preoperative care included pharmacologic adrenergic receptor blockade and fluid administration, guidelines for surgical readiness did not follow those recommended in our literature. Both patients experienced severe intraoperative hypotension after complete interruption of the tumors' venous drainage, and one patient suffered a cardiac arrest. Explanations for the occurrence of these problems are discussed, including factors relating to the complexity of the disease process. However, it is conceivable that appropriate input from the anesthesiologist during the preoperative preparation of these patients may have ameliorated, if not prevented, the encountered difficulties.
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3/22. mercury intoxication and arterial hypertension: report of two patients and review of the literature.

    Two children in the same household with symptomatic arterial hypertension simulating pheochromocytoma were found to be intoxicated with elemental mercury. The first child was a 4-year-old boy who presented with new-onset seizures, rash, and painful extremities, who was found to have a blood pressure of 171/123 mm Hg. An extensive investigation ensued. Elevated catecholamines were demonstrated in plasma and urine; studies did not confirm pheochromocytoma. mercury levels were elevated. These findings prompted an evaluation of the family. A foster sister had similar findings of rash and hypertension. Both had been exposed to elemental mercury in the home. The family was temporarily relocated and chelation therapy was started. A medline search for mercury intoxication with hypertension found 6 reports of patients ranging from 11 months to 17 years old. All patients showed symptoms of acrodynia. Because of the clinical presentation and the finding of elevated catecholamines, most of the patients were first studied for possible pheochromocytoma. Subsequently, elevated levels of mercury were found. Three children had contact with elemental mercury from a broken thermometer, 2 had played with metallic mercury and 1 had poorly protected occupational exposure. All responded to chelation therapy. Severe systemic arterial hypertension in infants and children is usually secondary to an underlying disease process. The most frequent causes of hypertension in this group include renal parenchymal disease, obstructive uropathy, and chronic pyelonephritis associated with reflux and renal artery stenosis. Less frequent causes include adrenal tumors, pheochromocytomas, neurofibromas, and a number of familial forms of hypertension. Other causes include therapeutic and recreational drugs, notably sympathomimetics and cocaine, and rarely, heavy metals. In children with severe hypertension and elevated catecholamines, the physician should consider mercury intoxication as well as pheochromocytoma. The health hazards of heavy metals need to be reinforced to the medical profession and the general public.
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4/22. Three patients with adrenal tumors having been treated simply for diabetes mellitus.

    Three patients with functional adrenal tumors, Cushing's syndrome, primary aldosteronism and pheochromocytoma, who underwent adrenalectomy and were subsequently cured, were studied. All these patients had been treated for diabetes for several years before the diagnosis of adrenal tumors. In each case the state of diabetes before and after surgery, including parameters of insulin secretion and insulin resistance, was compared to demonstrate how the adrenal disorder influenced the nature of diabetes. In the case of Cushing's syndrome the hypercortisolemia caused insulin resistance in the peripheral tissues. In the case of primary aldosteronism, excessive production of aldosterone diminished insulin secretion possibly through hypokalemia. pheochromocytoma affected both insulin secretion and insulin sensitivity through hypersecretion of catecholamines. In all these patients the adrenal tumors were found in clinical contexts other than management of diabetes itself. By careful retrospective review of these three patients' history, several important points that might have drawn the physician's attention to the underlying adrenal disorders were pointed out. These included past history of acute myocardial infarction with onset at unexpectedly young age in the case of Cushing's syndrome and unexpectedly high insulin resistance for the patient's body mass index in the case of pheochromocytoma.
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5/22. A case of giant malignant phaeochromocytoma.

    Malignant phaeochromocytoma is defined as the presence of tumour deposits at sites that are normally devoid of chromaffin cells. We report on a 63-year-old man who had a giant malignant phaeochromocytoma of the right adrenal gland that encased the inferior vena cava. The urinary excretion rates of catecholamines and their metabolites were normal, except for normetanephrine, which was excreted at a higher rate than normal. The tumour was surgically unresectable by laparotomy. Postoperatively, the patient was given a 4-month trial of subcutaneous octreotide and intravenous meta-iodobenzylguanidine I 131. Occult lung secondary tumours were first detected by meta-iodobenzylguanidine scintigraphy after 2 years, and the patient died of bone and lung metastases 1 year later. Because phaeochromocytoma is rare, local experience in managing this disease is limited. This report alerts physicians of the methods of diagnosing and managing surgically unresectable malignant phaeochromocytoma.
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6/22. pheochromocytoma.

    Causes of hypertension that are amenable to surgical treatment constitute a very small but potentially important segment of the hypertensive population. These causes (which constitute frequently asked questions for medical students) include coarctation of the aorta, aldosterone and corticosteroid-producing tumors of the adrenal glands, lesions producing decreased renal blood flow, and pheochromocytoma. This latter tumor is quite uncommon, with a frequency of roughly one per million, but often produces dramatic hypertensive episodes. Due to its rarity, physicians in practice may not consider the diagnosis and appropriately evaluate the patient. We present the case histories of three patients with pheochromocytoma who demonstrate important features of this disease. Diagnostic evaluation and principles of treatment will be discussed.
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7/22. Congenital hemihypertrophy and pheochromocytoma, not a coincidental combination?

    We describe a 19-year-old female, known to have congenital hemihypertrophy, who presented with bilateral benign pheochromocytoma. This is the second time that this combination has been reported in the literature. We speculate that the combination of congenital hemihypertrophy and pheochromocytoma is not coincidental and could be part of the clinical spectrum of the beckwith-wiedemann syndrome. CONCLUSION: in patients with congenital hemihypertrophy, the physician should be aware of the symptoms of pheochromocytoma. Besides screening for abdominal tumours, analysis of plasma and/or urinary catecholamines and/or their metabolites should be considered.
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8/22. Paroxysmal hypertension, pheochromocytoma, and pregnancy.

    BACKGROUND: hypertension is the most common medical complication of pregnancy. pheochromocytoma in pregnancy is rare, and if unrecognized, can cause serious perinatal morbidity and mortality. methods: A patient with severe hypertension, postpartum pulmonary edema, and a recognized pheochromocytoma is described. RESULTS: Abdominal palpation after vaginal childbirth reproduced the diagnostic triad of hypertension, headaches, and palpitations. magnetic resonance imaging established the correct diagnosis before biochemical confirmation of excess catecholamine production. The patient responded to alpha-adrenergic receptor blockade with control of her severe hypertension and clearing of pulmonary edema. The best time to diagnose a pheochromocytoma is before delivery because vaginal childbirth stimulates the release of lethal amounts of catecholamines. CONCLUSIONS: The physician who delivers babies must distinguish between labile hypertension and paroxysmal hypertension. Most experts believe that a spontaneous vaginal delivery is contraindicated when the patient has a pheochromocytoma. Postpartum pulmonary edema associated with a pheochromocytoma is unusual. The profound pressor response elicited by palpation of the postpartum abdomen, the failure of medications usually effective in the treatment of a hypertensive crisis, and the use of magnetic resonance imaging to confirm a functioning adrenal adenoma are the features unique to this case.
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9/22. Phaeochromocytoma by way of case reports.

    Endocrine causes of hypertension are relatively rare, but their detection offers a real chance for cure. This is particularly true of phaeochromocytoma, a catecholamine-producing tumour derived from chromaffin tissue, which, if left undetected, is invariably fatal. The lethal nature of this tumour is dependent on two major characteristics: firstly, its ability to secrete catecholamines in excess, resulting in potentially catastrophic consequences; and, secondly, its malignant potential. Approximately 5-10% of these tumours are malignant, which, if metastasised, are generally refractory to treatment. Clearly, however, because only one in 1,000 hypertensives is likely to harbour a phaeochromocytoma, it is not cost-effective option to screen all hypertensives for this cause. Rather, a selective approach is preferred in which a high index of suspicion for the clinical characteristics of this tumour is used to guide the physician. The following two case reports derived from our records at Chris Hani Baragwanath Hospital will illustrate many issues related to diagnosis and management of this fascinating tumour.
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10/22. Interesting clinical case studies involving secondary causes of hypertension.

    While essential hypertension is very prevalent, secondary hypertension does occur in clinical practice with sufficient frequency to warrant special attention being given to those patients with severe hypertension who do not respond to standard treatments. The evaluation, diagnosis and treatment of secondary hypertension pose interesting challenges to the physician. In this article, three patients are described with different secondary causes of hypertension.
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