Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 42-year-old female who presented with retrosternal pain, dyspnoea and nausea. Electrocardiography suggested a recent anterior myocardial infarction. However, emergency coronary angiography showed normal blood flow through all the coronary arteries. Paroxysmal hypertension raised the suspicion of a pheochromocytoma. Indeed, abdominal ultrasonography and computed tomography revealed a mass in the left adrenal gland. Elevated levels of plasma and urine catecholamines supported the diagnosis of pheochromocytoma. Left adrenalectomy was performed without complications and pathological examination revealed a 5.5 cm pheochromocytoma. After surgery, all antihypertensive medication was discontinued and the blood pressure returned to normal within several days. Currently, the patient is asymptomatic, has normal catecholamine levels and the electrocardiographic signs of ischaemia have resolved entirely. This case illustrates that a rare clinical entity such as pheochromocytoma should be considered in the differential diagnosis of acute coronary syndrome. (Neth Heart J 2007;15:248-51.).
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PMID:Pheochromocytoma mimicking an acute myocardial infarction. 1792 79

Paroxysmal hypertension always engenders a search for a catecholamine-secreting pheochromocytoma. Yet 98% of people with paroxysmal hypertension do not have this tumor. The cause and management of paroxysmal hypertension remain a mystery, and the subject of remarkably few papers. This review presents an approach to understanding and successfully treating this disorder. Patients experience symptomatic blood pressure surges likely linked to sympathetic nervous system stimulation. A specific personality profile associated with this disorder suggests a psychological basis, attributable to repressed emotion related to prior emotional trauma or a repressive (nonemotional) coping style. Based on this understanding, three forms of intervention, alone or in combination, appear successful: antihypertensive therapy with agents directed at the sympathetically mediated blood pressure elevation (eg, combined alpha- and beta-blockade or central alpha-agonists such as clonidine); psychopharmacologic interventions including anxiolytic and/or antidepressant agents; and psychological intervention, particularly reassurance and increased psychological awareness. An appropriately selected intervention can reduce or eliminate attacks in most patients.
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PMID:Severe paroxysmal hypertension (pseudopheochromocytoma). 1836 21

Pheochromocytomas (PCCs) are rare catecholamine producing neuroendocrine tumors. The majority of these tumors (85 %) arise from the adrenal medulla. Those arising from the extra-adrenal neural ganglia are called paragangliomas (PGLs). Paroxysmal hypertension with sweating, headaches and palpitation are the usual presenting features of PCCs/ PGLs. Gene mutations are reported in 32-79 % of cases, making genetic screening mandatory in all the cases. The malignancy rates are 10-15 % for PCCs and 20-50 % for PGLs. Measurement of plasma or 24-hour urinary fractionated metanephrines is the best biochemical diagnostic test. Computed tomography or magnetic resonance imaging has high sensitivity (90-100 %) and reasonable specificity (70-90 %) for the anatomical localization. The functionality is assessed by different radionuclide imaging modalities such as metaiodobenzylguanidine (MIBG) scintigraphy, positron emission tomography or single photon emission computed tomography. The only modality of curative treatment is tumor excision. Proper peri-operative management improves the surgical outcomes. Annual follow up with clinical and biochemical assessment is recommended in all the cases after treatment. Children, pregnant women and older people have higher morbidity and mortality risk. De-bulking surgery, chemotherapy, radiotherapy, molecular agents like sunitinib and everolimus, radionuclide agents and different ablation procedures may be useful in the palliation of inoperable/metastatic disease. An update on the diagnostic evaluation and management of PCCs and PGLs is presented here.
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PMID:Diagnosis and management of pheochromocytoma: a practical guide to clinicians. 2479 93

Although "labile hypertension" is regularly encountered by clinicians, there is a paucity of information available to guide therapeutic decisions. This review discusses its clinical relevance, the limitations of current knowledge, and possible directions for future research and clinical management. Results of studies that assessed measures of blood pressure variability or reactivity are reviewed. The limited information about effects of antihypertensive drugs on blood pressure variability is discussed. Two different clinical presentations are differentiated: labile hypertension and paroxysmal hypertension. Labile hypertension remains a clinical impression without defined criteria or treatment guidance. Paroxysmal hypertension, also called pseudopheochromocytoma, presents as dramatic episodes of abrupt and severe blood pressure elevation. The disorder can be disabling. Although it regularly raises suspicion of a pheochromocytoma, such a tumor is found in <2 % of patients. The cause, which involves both emotional factors and the sympathetic nervous system, and treatment approaches, are presented.
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PMID:Labile and Paroxysmal Hypertension: Common Clinical Dilemmas in Need of Treatment Studies. 2637 May 55

Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed. Abdominal ultrasonography revealed a right adrenal gland tumor. Fractionated catecholamines and metanephrines in plasma and 24-h collected urine revealed elevated levels of norepinephrine and normetanephrine. Although hypertension and tachycardia were inapparent by an ordinary physical examination, paroxysmal mild hypertension and tachycardia were identified by a thorough examination after walking and abdominal compression. Paroxysmal hypertension and tachycardia were profound during operation. In conclusion, pheochromocytoma can be a consideration in the differential diagnosis of weight loss. Hypertension and tachycardia can be inapparent and paroxysmal in pediatric patients as well as in adults; thus, thorough assessment should be repeated.
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PMID:A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease. 2966 68

Four cases of mixed neuroendocrine-neural tumors composed of pheochromocytoma and neuroblastoma elements (including ganglioneuroma and ganglioneuroblastoma) were studied for the presence of catecholamine-synthesizing enzymes, neuroendocrine markers, and peptide hormones with clinicopathological correlations. Paroxysmal hypertension with hypercatech olaminemia was observed in 3 patients. One patient had an extremely elevated level of dopamine. The location of the tumor was in the adrenal glands in 2 patients and in the retroperitoneum in the other 2. Numerous electron-dense granules in the cytoplasm and neural processes with abundant neurotubules were characteristic of mixed neuroendocrine-neural tumors. Immunohistochemical study revealed that catecholamine-synthesizing enzymes were present in both components of the pheochromocytoma and neuroblastoma group, but phenylethanolamine N-methyltransferase was detectable only in epinephrine-producing tumors. Chromogranin and neurofilament immunoreactivities were present in both components; however, the intensity of chromogranin immunoreactivity was stronger in pheochromocytoma than in the other components. In contrast, neurofilament positivity was stronger in the neuroblastoma group than it was in pheochromocytoma. Multiple peptide hormones were immunoreactive in both components. Neuropeptide Y and met-enkephalin-positive cells were numerous in both; cells containing vasoactive intestinal peptide and somatostatin were less common but were comparatively more frequently found in ganglion cells than in pheochromocytoma cells.
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PMID:Adrenal and retroperitoneal mixed neuroendocrine-neural tumors. 3235 42

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity that is manifested by characteristic magnetic resonance imaging (MRI) depictions of subcortical/cortical hyperintensities in the parieto-occipital lobes. Paroxysmal hypertension, headache, and palpitation are the most common clinical manifestations of pheochromocytoma, which are catecholamine-secreting enterochromaffin tumors. PRES is a rare complication of pheochromocytoma. Herein, we describe a 44-year-old woman who presented with postoperative confusion and headache. MRI images showed multiple asymmetrical hyperintensities with surrounding edema and contrast enhancement, predominantly in the right parietal lobe, left cerebellar hemisphere, and dentate nuclei, in favor of hemorrhagic metastases. The results of further investigations, including abdominopelvic computed tomography and the 24-hour urine test for metanephrine and normetanephrine, were in favor of a pheochromocytoma. The patient was scheduled for adrenalectomy and histopathologic examination of the tissue, which confirmed the diagnosis. Surprisingly, her symptoms and neuroimaging abnormalities improved significantly without any treatment during the follow-up period. Based on these findings, the diagnosis of PRES was considered, and the patient was followed. She was symptom-free at 3 years' follow-up. The literature contains only four case reports of PRES as a complication of pheochromocytoma; however, all these cases had bilateral symmetrical hemispheric involvement and occurred during childhood and adolescence.
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PMID:Atypical Posterior Reversible Encephalopathy Syndrome as the First Presentation of a Pheochromocytoma: A Case Report. 3328 Dec 66