Comprehensive review of evaluation and management of cardiac paragangliomas

Abstract

Cardiac paraganglioma (PGL) is a rare neuroendocrine tumour causing significant morbidity primarily due to norepinephrine secretion potentially causing severe hypertension, palpitations, lethal tachyarrhythmias, stroke and syncope. Cardiologists are faced with two clinical scenarios. The first is the elevated norepinephrine, whose actions must be properly counteracted by adrenoceptor blockade to avoid catastrophic consequences. The second is to evaluate the precise location of a cardiac PGL and its spread since compression of cardiovascular structures may result in ischaemia, angina, non-noradrenergic-induced arrhythmia, cardiac dysfunction or failure. Thus, appropriate assessment of elevated norepinephrine by its metabolite normetanephrine is a gold biochemical standard at present. Furthermore, dedicated cardiac CT, MRI and transthoracic echocardiogram are necessary for the precise anatomic information of cardiac PGL. Moreover, a cardiologist needs to be aware of advanced functional imaging using 68Ga-DOTA(0)-Tyr(3)-octreotide positron emission tomography/CT, which offers the best cardiac PGL-specific diagnostic accuracy and helps to stage and rule out metastasis, determining the next therapeutic strategies. Patients should also undergo genetic testing, especially for mutations in genes encoding succinate dehydrogenase enzyme subunits that are most commonly present as a genetic cause of these tumours. Curative surgical resection after appropriate α-adrenoceptor and β-adrenoceptor blockade in norepinephrine-secreting tumours is the primary therapeutic strategy. Therefore, appropriate and up-to-date knowledge about early diagnosis and management of cardiac PGLs is paramount for optimal outcomes in patients where a cardiologist is an essential team member of a multidisciplinary team in its management.

Giant Paraganglioma Complicated With Catecholamine Crisis and Catecholamine Cardiomyopathy: A Case Report and Review of the Literature

“…About 10% of PPGL are metastatic, and they usually relate to germline mutations in the gene encoding succinate dehydrogenase complex iron sulfur subunit B (SDHB). As most PPGL are benign, surgical resection is the optimal method, and the recovery and prognosis are quite good after complete tumor resection (16).…”

Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China

“…Genetic testing is recommended for all PGL patients, not only because genetic diagnosis provides important information for clinical management, prognosis, and translational research, but also to increase understanding of the hereditary nature of these tumors. Currently, 22 genes have been linked to the pathogenesis of PGLs, and 30% to 40% of PGLs are hereditary (23)(24)(25)(26). Among CPGL patients, mutations in SDHB and SDHD have been most commonly reported; mutations in PGL-associated genes such as the rearranged during transfection and von Hippel-Lindau genes have not been detected (6).…”

Cardiac Paraganglioma, Recent Advances in Clinicopathologic Features

“…A 2-fold increase in metanephrine levels above the upper limit is highly suggestive of PGs. However, small PGs and SDH-mutated PGs may not reach this threshold due to their lower catecholamine contents [22]. Although tissue biopsy is essential for the diagnosis, performing biopsy for the initial work-up is difficult and can result in bleeding complications due to the tumor's rich vascularity.…”

“…However, it should be kept in mind not to give a beta-blocker unless the alpha-blocker has successfully controlled the blood pressure. Otherwise, alpha-receptor stimulation could lead to a catecholamine storm and hypertensive crisis [22]. The first step before the surgical management of cardiac PGs is to determine whether the tumor is resectable or not.…”

“…The first step before the surgical management of cardiac PGs is to determine whether the tumor is resectable or not. Therefore, pre-operative assessment should include the tumor's location, the proximity of the tumor to cardiac structures and great vessels, and its extracardiac extension [22]. The common approach for tumor resection is sternotomy with cardiopulmonary bypass followed by the right or left thoracotomy [3].…”