Cardiac Paraganglioma

Abstract

Paragangliomas are extraadrenal tumors that arise from chromaffin cells of the sympathetic ganglia. Cardiac paraganglia are exceptionally rare and because of paucity of cohort data, raises dilemmas about management when encountered. Some of them are metabolically active and may present with symptoms of catecholamine excess, whereas some may be incidentally diagnosed. In this report, we discuss the challenges surrounding the diagnosis and management of a cardiac paraganglioma at each stage. Case PresentationA 73-year-old woman is referred by her cardiologist for an elective coronary angiography because of an abnormal stress test in the setting of dyspnea on exertion. The dyspnea started ≈6 months ago and has been slowly progressive. She continues to try to exercise through this and is currently able to walk 1 mile in 30 minutes, a pace that is slower than usual. The medical history is significant for mild edema (hydrochlorothiazide 25 mg/d), dyslipidemia (diet controlled); there is no history of smoking, hypertension, diabetes mellitus, coronary artery disease, or peripheral atherosclerosis. Her family history is notable for coronary artery disease.On physical examination, the patient's body mass index is 35 (5 ft 0 in; 81.6 kg), blood pressure is 140/70 mm Hg with a pulse of 60 bpm. Jugular venous pulse is normal. Carotid upstroke is brisk, and there are no bruits. Lungs are clear to auscultation. The first and the second heart sounds are normal; there are no murmurs, rubs, or gallops. There is no lower extremity edema, and rest of the examination is unremarkable. An ECG demonstrates normal sinus rhythm with possible anteroseptal infarct. A chest radiograph is normal.The stress echocardiography report shows that the patient exercised for 7:30 minutes on a Bruce protocol achieving a peak heart rate of 146 bpm (99% maximum predicted heart rate) and blood pressure of 180/60 mm Hg. The resting heart rate and blood pressure were 71 bpm and 118/60 mm , respectively. The resting ECG showed normal sinus rhythm with possible anteroseptal infarct, and the stress ECG showed sinus tachycardia with nonspecific ST-T wave changes. The resting part of stress echocardiography showed normal wall motion, and with exercise, the left ventricle became smaller and more vigorous, but the anterior septum and septum failed to become hyperkinetic, and therefore the study was read as an abnormal study. The patient was then referred to a cardiologist for further evaluation. Certainly, the stress test abnormality was not high risk, and a trial of medical therapy with investigation of alternate pathogenesis for dyspnea versus diagnostic coronary angiography was discussed. However, the patient's symptoms were out of proportion to the degree of stress test abnormality, with no other obvious pathogenesis. The patient was concerned about ischemic heart disease in light of not a normal stress test, and decision was made to proceed with a diagnostic coronary angiography.At this time, the patient undergoes coronary angiography via transradial access...

Unusual cardiac paraganglioma mimicking an atypical carcinoid tumor of the lung

“…An additional 2.5% mortality was reported for the immediate postoperative course. A review of individual case reports from 2014-2016, two years since Wang et al's review, shows no intraoperative mortality reported from 11 cases (12,18,19,(22)(23)(24)(25)(26)(27). Among these cases, one instance of intraoperative cardiac arrest occurred due to severe hemorrhage and one unexpected intraoperative circulatory collapse, however both ended with successful outcomes (24,26).…”

“…After recovery from surgery, benign cardiac PGs that are completely resected with negative margins have been associated with favorable outcomes in survival with low rates of tumor recurrence (18,26). For those surviving the immediate post-operative course, a 1-year survival of 98.2% and 5-year survival of 78.2% has been reported (17).…”

Utility of 18F-Fluorodeoxyglucose positron emission tomography/magnetic resonance imaging in the diagnosis of cardiac paraganglioma

“…[ 3 ] Clinically, these can present without symptoms or with generalized complaints and signs of catecholamine excess. [ 4 ] The extreme variability in symptoms suggests a broad differential, which necessitates further investigation. There are many imaging options available, with no standard consensus on the most appropriate studies.…”

Cardiac Paraganglioma, Recent Advances in Clinicopathologic Features

“…Imaging studies have improved over the last few decades and have become very sensitive and specific for diagnosing and localizing cardiac PGs. Commonly used modalities include contrastenhanced computed tomography (CT) scan, dedicated cardiac magnetic resonance imaging (MRI), transthoracic echocardiogram (TTE), and coronary arteriography [23]. By echocardiography, cardiac PGs appear as large, echogenic masses [4].…”

“…CT scan will show heterogeneous mass with peripheral enhancement and low attenuation areas due to tumor degeneration and necrosis [24]. By MRI, cardiac PGs are shown as well-circumscribed ovoid masses with hyperintense signals on the T2 imaging sequence [23]. Cardiac MRI is superior to CT scan and echocardiography for detection and evaluation of cardiac PGs [1].…”