Secondary hypertension: An update on the diagnosis and localisation of a pheochromocytoma or paraganglioma (original) (raw)
Related papers
Endocrine, 2014
Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1-0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as *30 % of subjects are normotensive or have orthostatic hypotension and in these cases the 24 h ambulatory blood pressure (BP) monitoring is an important toll to diagnose and treat HT. HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective a1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a b-adrenergic blockade (i.e., propranolol, atenolol). This latter should never be started first because blockade of vasodilatory peripheral b-adrenergic receptors with unopposed a-adrenergic receptor stimulation can lead to a further elevation of BP. Although labetalol is traditionally considered the ideal agent due to its aand b-adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.
Pheochromocytoma: A Rare Cause of Secondary Hypertension
Journal of Lumbini Medical College, 2013
Introduction: Pheochromocytoma is a rare tumor, originating from the chromaffin tissue. Its frequency is approximately 1/100,000. The clinical manifestation is due to catecholamine excess, which includes high blood pressure, palpitation, headache, sweating, nausea, vomiting, trembling, weakness, irritation, abdominal and chest pain, dyspnea, red warm face, constipation, polyuria, and polydipsia. Case Report: We present a case of 53 years old male, hospitalized for hypertensive crisis following the manipulation of left sided frozen shoulder. He had labile blood pressure ranging from 220/120 systolic- 90/60 diastolic, profuse sweating and tachycardia. Findings of Contrast enhanced CT of abdomen was consistent with right adrenal pheochromocytoma and 24 hours urinary VMA was 17 mg /24 hr (Normal <13.6 mg /24hr). After the clinical, paraclinical investigations and radiological tests, it was proved to be a pheochromocytoma. The surgical intervention was planned. But due to unavaila...
Pheochromocytoma and paraganglioma: from clinical findings to diagnosis
SiSli Etfal Hastanesi Tip Bulteni / The Medical Bulletin of Sisli Hospital, 2020
The majority of pheochromocytoma (PCC) and paraganglioma (PGL) are endocrine active tumors, and they cause clinical symptoms by secreting excess one or more catecholamines (epinephrine, norepinephrine, and dopamine) and their inactive metabolites (metanephrine, normetanephrine, 3-metoxythromine). Although signs and symptoms regarding excess catecholamine often develop in PCC and PGL (PPGL), non-functional PPGLs may present with local compression symptoms. Persistent, sometimes worsening hypertension is the most common finding and occurs in 80-90% of the patients. Classically defined symptom triad; headache, sweating and palpitations are seen in only 25% of the patients with PCC. The difference of clinical symptoms may be related to the tumor secretion, epinephrine or norepinephrine. All patients with signs and symptoms suggestive of catecholamine excess should be screened by biochemical tests regardless of whether they have hypertension or not. Not all patients with newly diagnosed hypertension need to be screened, but only patients with additional tips for catecholamine excess should be screened. Approximately 20% of the PPGLs are diagnosed in childhood, and the male/female ratio is 2/1. 60-90% of pediatric patients present with hypertension. PPGL in pregnancy is rare, and the estimated incidence ranges between 1/15000-1/54000. Although early diagnosis is the most important factor in preventing mortality, diagnosis is not as easy as it is a rare condition. Hypertension is a common complication in pregnancy, occurring in 5-10%. Computed tomography should not be used as the imaging method during pregnancy; the first choice is magnetic resonance imaging with gadolinium or without contrast. Plasma free metanephrine or 24-hour urinary fractionated metanephrine level is recommended as a screening test for the diagnosis of PPGL in the Endocrine Society Clinical Practice Guideline. In suspicious situations, tests should be repeated. Since 40% of these patients have germline mutations, genetic tests are recommended for all patients with PPGL regardless of family history and age. Preoperative knowledge of germline mutations affects the surgical approach and the extent of adrenalectomy. After the biochemical diagnosis is made in PPGL, the tumor is localized with imaging methods to make the operation plan. In this review, we aimed to evaluate the clinical findings, diagnostic tests, and imaging studies for tumor localization in PPGL.
Cardiovascular Manifestations and Complications of Pheochromocytomas and Paragangliomas
Journal of Clinical Medicine
Pheochromocytomas and paragangliomas (PPGLs) are rare neuro-endocrine tumors. The catecholamine surge causes paroxysmal or chronic secondary hypertension. PPGLs may present as hypertensive- or PPGL-crisis with severe life-threatening cardiac and cerebrovascular complications. PPGLs-induced cardiac manifestations have been reported with diagnoses as PPGLs-induced electrocardiogram (ECG) changes “mimicking acute myocardial infarction”, arrhythmias, myocarditis, acute coronary syndrome, dilated cardiomyopathy, and lately as takotsubo syndrome. Critical analysis of these reports reveals that most of these cardiac manifestations have certain features in common. They have a dramatic clinical presentation and are reversible if the disease is treated with appropriate medical therapy and surgical resection of the PPGL tumor. They may have the same repolarization ECG changes irrespective of the clinical cardiac diagnosis, usually associated with mild to moderate elevations of myocardial bioma...
Pheochromocytoma: A Rare Culprit of Uncontrolled Hypertension
2019
Pheochromocytoma is a rare cause of hypertension that is curable. We present a case report of a 24-year-old female who presented with severely elevated blood pressure and palpitations. Investigations revealed a left adrenal mass that was successfully excised. A high index of suspicion is required to make a diagnosis of pheochromocytoma in young patients with severely elevated blood pressure.
Pheochromocytoma: A Rare Cause of Hypertension
IOSR Journals , 2019
I hereby report a case of pheochromocytoma, rare cause for hypertension. Pheochromocytomas are rare tumours arising from adrenal medulla and it can present with abdominal pain and headache. A 35 year old lady with no co-morbidities presented with complaints of abdominal pain confined to right lumbar region with headache since 3 months ; low grade fever of 4 days. On examination Pulse =116/ min , normal volume , Blood Pressure = 150/110 mm of Hg. Per abdomen no palpable mass .Radiologically diagnosed as right suprarenal mass ,which is confirmed by laboratory investigations and upon adrenalectomy operation and histopathology examination diagnosed as Pheochromocytoma.
Secondary hypertension: A rare cause
Indian Journal of Endocrinology and Metabolism, 2011
A 13-year-old, previously asymptomatic girl was admitted with features of tuberculous meningitis. She was found to be hypertensive and further investigations revealed an extra-adrenal paraganglioma. Tuberculous meningitis and paraganglioma could be chance associations. Paraganglioma is a very rare and potentially lethal cause of secondary hypertension. We are reporting a very rare disease, which has come to light in a most unexpected manner.
Unexplained Symptomatic Paroxysmal Hypertension in Pseudopheochromocytoma
Annals of the New York Academy of Sciences, 2008
Among overall numbers of patients tested for pheochromocytoma, less than 2% harbor the tumor. Among the rest, there is often no satisfactory explanation for the signs and symptoms leading to suspicion of pheochromocytoma. This group includes patients with severe symptomatic paroxysmal hypertension, often referred to as pseudopheochromocytoma, a condition that can be debilitating for patients and perplexing for clinicians. Similar to patients with the real tumor, patients with pseudopheochromocytoma can be misdiagnosed with panic disorder. However, pseudopheochromocytoma is characterized by an absence of panic or emotional distress preceding the onset of hypertension and symptoms of catecholamine excess. Because the clinical manifestations of pseudopheochromocytoma are similar, if not identical, to those due to excess circulating catecholamines in patients with the tumor, the most attractive explanation for the disorder is that it involves altered function of the autonomic nervous system. In line with this hypothesis, recent findings suggest that enhanced adrenal release of epinephrine and exaggerated cardiovascular responsiveness to catecholamines both contribute to the paroxysmal hypertension and symptoms of catecholamine excess in pseudopheochromocytoma. From this pattern, one would predict that therapeutic interventions that inhibit adrenal secretion of epinephrine or block adrenoceptor-mediated responses to catecholamines might provide a logical approach to therapy.