Cardiac Paraganglioma: Multimodality Imaging of a Rare Tumor

Abstract

Paragangliomas are rare tumors of chromaffin cells arising from an extra-adrenal location. Unlike pheochromocytomas, they are seldom functional. We present a case of pericardial paraganglioma incidentally encountered on an echocardiographic study, focusing on the characteristic features the tumor demonstrates on different imaging modalities. (Level of Difficulty: Intermediate.).

Keywords: computed tomography; coronary angiography; echocardiography; nuclear medicine imaging.

Graphical abstract

Figure 1

Multimodality Imaging of a Pericardial Paraganglioma (A) Suprasternal echocardiographic view, where under the aortic arch and the right pulmonary artery, an echogenic mass was found and on color Doppler imaging with low-velocity scale appears as a richly perfused blood mass with continuous flow of the feeding arteries (a). Yellow arrows show the mass. (B) Computed tomography reveals a well-circumscribed mass under the ascending aorta and the right pulmonary artery. Yellow arrows show the mass. (C) Scintigraphic images with technetium-99m tektrotyd revealed an increased uptake of the radiotracer in the anatomical region between the left and right atrium of the heart and under the right pulmonary artery, confirming a mass with neuroendocrine characteristics (increased expression of somatostatin receptors 2, 3, 5). (D) Scintigraphic images with iodine-123 metaiodobenzylguanidine scan confirms that the mass possesses adrenergic (increased uptake of odine-123 metaiodobenzylguanidine) characteristics. (E, F) Selective angiographies of the (E) left and (F) right coronary arteries, demonstrating the feeding arteries of the mass, arising from both the right and the left circumflex (CMX) coronary artery. 99mTc = technitium-99m; Ao = aorta; LAD = left anterior descending coronary artery; MIBG = metaiodobenzylguanidine; PA = pulmonary artery; RCA = right coronary artery; RPA = right pulmonary artery.