Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension

Lenders, Jacques W.M.; Kerstens, Michiel N.; Amar, Laurence; Prejbisz, Aleksander; Robledo, Mercedes; Taïeb, David; Pacák, Karel; Crona, Joakim; Zelinka, Tomáš; Mannelli, Massimo; Deutschbein, Timo; Timmers, Henri J L M; Castinetti, Frédéric; Dralle, Henning; Widimský, J; Gimenez‐Roqueplo, Anne‐Paule; Eisenhofer, Graeme

doi:10.1097/hjh.0000000000002438

Cited by 259 publications

(419 citation statements)

References 130 publications

(206 reference statements)

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“…Based on the applied diagnostic and statistical analyses we found a good diagnostic performance with the area under curves of 0.86 and 0.83, respectively. Since the diagnostic performance of the currently recommended selection criteria for EHT screening—such as young age at diagnosis or high grade or resistant hypertension ( 14 , 15 , 29 )—is not available, a direct comparison with current approaches is not possible. However, considering only the prevalence of PA, as the most common EHT of up to 23% in patients with uncontrolled hypertension, our preselection approach might be more selective in identifying EHT cases, with a specificity of up to 45% while maintaining a reasonably high sensitivity of 80% ( Fig.…”

Section: Discussionmentioning

confidence: 99%

Targeted Metabolomics as a Tool in Discriminating Endocrine From Primary Hypertension

Erlic

Reel

et al. 2020

The Journal of Clinical Endocrinology &Amp; Metabolism

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Context Identification of patients with endocrine forms of hypertension (EHT) (primary hyperaldosteronism [PA], pheochromocytoma/paraganglioma [PPGL] and Cushing syndrome [CS]) provides the basis to implement individualized therapeutic strategies. Targeted metabolomics (TM) have revealed promising results in profiling cardiovascular diseases and endocrine conditions associated with hypertension. Objective Use TM to identify distinct metabolic patterns between primary hypertension (PHT) and EHT and test its discriminating ability. Design Retrospective analyses of PHT and EHT patients from a European multicentre study (ENSAT-HT). TM was performed on stored blood samples using liquid chromatography mass spectrometry. To identify discriminating metabolites a “classical approach” (CA) (performing a series of univariate and multivariate analyses) and a “machine learning approach” (MLA) (using Random Forest) were used. Patients The study included 282 adult patients (52% female; mean age 49 years) with proven PHT (n=59) and EHT (n=223 with 40 CS, 107 PA and 76 PPGL), respectively. Results From 155 metabolites eligible for statistical analyses, 31 were identified discriminating between PHT and EHT using the CA and 27 using the MLA, of which 15 metabolites (C9, C16, C16:1, C18:1, C18:2, arginine, aspartate, glutamate, ornithine, spermidine, lysoPCaC16:0, lysoPCaC20:4, lysoPCaC24:0, PCaeC42:0, SM C18:1, SM C20:2) were found by both approaches. The ROC curve built on the top 15 metabolites from the CA provided an area under the curve (AUC) of 0.86, which was similar to the performance of the 15 metabolites from MLA (AUC 0.83). Conclusions TM identifies distinct metabolic pattern between PHT and EHT providing promising discriminating performance.

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Section: Discussionmentioning

confidence: 99%

Targeted Metabolomics as a Tool in Discriminating Endocrine From Primary Hypertension

Erlic

Reel

et al. 2020

The Journal of Clinical Endocrinology &Amp; Metabolism

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“…Surgical resection of the tumor is the only curative option [1,118]. Preoperative management consists of alpha blockade and correction of intravascular volume [5,24,27].…”

Section: Ppgl Managementmentioning

confidence: 99%

“…The medical treatment before surgery is almost entirely based on expert opinion and observational studies. Treatment with alpha adrenergic receptor blocker is considered as the treatment of choice [118]. The two most commonly used drugs are phenoxybenzamine, which is a nonselective and noncompetitive alpha-1 and alpha-2 adrenergic receptor blocker.…”

Section: Ppgl Managementmentioning

confidence: 99%

“…In an acute situation, PPGL-crisis alpha-blockers in the form of intravenous phentolamine is preferred over phenoxybenzamine because it has a rapid action. Calcium channel blockers (nifedipine or amlodipine) have also been used and preferably added to alpha adrenergic receptor blocker for further improvement of blood pressure control [118]. Beta-blockers are indicated to control tachycardia.…”

Section: Ppgl Managementmentioning

confidence: 99%

“…Beta-blockers in the form propranolol, metoprolol or atenolol can be used. Evidence to support the preference of beta-1 selective blockers over non-selective betablockers does not exist [1,118]. Labetalol with its more potent beta-than alpha-antagonist activities is not recommended as the initial therapy because it can lead to paradoxical hypertension and even hypertensive crisis [1].…”

Section: Ppgl Managementmentioning

confidence: 99%

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Cardiovascular Manifestations and Complications of Pheochromocytomas and Paragangliomas

Y‐Hassan

Falhammar

2020

JCM

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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 biomarkers as troponins and normal coronary arteries. The histopathological findings are usually focal or multifocal in the form hypercontracted sarcomeres and contraction band necrosis (myofibrillar degeneration) with subsequent secondary mononuclear cell infiltration. Evidences argue the PPGL caused surge of catecholamines triggers hyperactivation of the sympathetic nervous system with cardiac sympathetic nerve terminal disruption with norepinephrine spillover causing the cardiac complications. A comprehensive review of various reported cardiovascular manifestations and complications of PPGLs are presented.

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Caring for Patients With Pheochromocytomas in the Perioperative Setting

Criscitelli

2024

AORN Journal

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Pheochromocytomas are rare, complex catecholamine‐secreting tumors of the adrenal medulla. Patients with pheochromocytomas may experience episodes of life‐threatening hypertension, and untreated tumors can be fatal. The nonspecific signs and symptoms (eg, headaches, palpitations, sweating) make diagnosing these tumors difficult. A comprehensive patient history, clinical evaluation during episodic hypertension, and biochemical test results can facilitate a definitive diagnosis. The only curative treatment is minimally invasive tumor removal. Because the tumor may recur, patients require follow‐up visits for approximately 10 years after surgery. Preoperative evaluations can include cardiac and imaging studies and genetic testing. Preoperative preparation involves controlling hypertension and planning for hypotension after tumor removal. Perioperative nurses should be prepared to assist anesthesia professionals with placing invasive hemodynamic monitors and responding to blood pressure changes. An interdisciplinary team can create a guideline or protocol for nurses to follow to enhance outcomes when caring for patients with pheochromocytomas.

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Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension

Abstract: Significant differences between patients with and without PPGL. c Symptom score: one point for each sign: BMI < 25 kg/m 2 , heart rate !85 beats/min, pallor, sweating, palpitations, tremor and nausea; a negative point for obesity.

Cited by 259 publications

References 130 publications

Targeted Metabolomics as a Tool in Discriminating Endocrine From Primary Hypertension

Targeted Metabolomics as a Tool in Discriminating Endocrine From Primary Hypertension

Cardiovascular Manifestations and Complications of Pheochromocytomas and Paragangliomas

Caring for Patients With Pheochromocytomas in the Perioperative Setting

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