Cardiac paragangliomas are extremely rare tumors that can be completely resected surgically, though postoperative complications from endogenous catecholamine release carry a significant mortality risk.
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Informs perioperative planning for these vascular tumors; leaves open standardized treatment algorithms.
All articles of this category Objectives: Cardiac paragangliomas are extremely rare and represent only 1% of all primary cardiac tumors. These are extra-adrenal pheochromocytomas arising from chromaffin cells. Methods: From June 2004 until August 2016, we evaluated retrospectively all patients with a diagnosis of primary cardiac tumors ( n = 82, 71 benign, 11 malign). Histological confirmation of paraganglioma was 3.66% ( n = 3). Preoperative all patients had transthoracal/transesophageal echocardiography, additionally MRI or CT-scan. In one case 3-D-printing was used for planning the operation. Results: The median age was 61.77 ± 14.38 years (2 male, 1 female). Preoperative symptoms were paroxysmal hypertension, palpitations and dyspnea. One was located paracardial, the others intracardial in the right atrium and right ventricle. The mean size of tumor was 3.13 ± 1.3 mm. In all 3 patients the paraganglioma could be completely removed by surgery with cardiopulmonary bypass. One patient died postoperative due to side effects of endogenous catecholamines (intestinal ischemia). The other 2 patients had a good outcome. Thus 30-day mortality was 33.33%. Conclusion: Cardiac paragangliomas are rare and difficult to be detected. Measurements for urine and serum catecholamines could help identify the type of the tumor. 3-D-Printing may help to plan the complex operation after diagnosis of cardiac tumors. Follow up by CT-scan and hormone screening is necessary to identify recurrence.
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Brendel et al. (2017) studied this question.
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