Intrapericardial Paraganglioma

HomeRadiology: Cardiothoracic ImagingVol. 4, No. 4 PreviousNext Images in Cardiothoracic ImagingFree AccessCardiac ImagingIntrapericardial ParagangliomaLucas de Pádua Gomes de Farias , Gustavo Borges da Silva Teles, Luciana de Pádua Silva Baptista, Andrei Skromov de AlbuquerqueLucas de Pádua Gomes de Farias , Gustavo Borges da Silva Teles, Luciana de Pádua Silva Baptista, Andrei Skromov de AlbuquerqueAuthor AffiliationsFrom the UnitedHealth Group Brazil, Hospital Samaritano, R. Conselheiro Brotero 1486, Higienópolis, São Paulo, SP 01232-010, Brazil (L.d.P.G.d.F., G.B.d.S.T., L.d.P.S.B.); and Fleury Medicina e Saúde, São Paulo, Brazil (L.d.P.G.d.F., A.S.d.A.).Address correspondence to L.d.P.G.d.F. (email: [email protected]).Lucas de Pádua Gomes de Farias Gustavo Borges da Silva TelesLuciana de Pádua Silva BaptistaAndrei Skromov de AlbuquerquePublished Online:Jul 28 2022https://doi.org/10.1148/ryct.220100MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In A 56-year-old man, who was asymptomatic, was referred for an elective coronary calcium score stratification. Non–contrast-enhanced cardiac CT scan (Fig 1A) showed a circumscribed nodule, measuring 2.6 cm, surrounded by the inferior border of aortic arch, the posterior border of ascending aorta, the upper border of the right main pulmonary artery, and the anterior border of the trachea and proximal left main bronchus. A subsequent contrast-enhanced chest CT scan (Fig 1B) showed an intense postcontrast enhancement and a heterogeneous nonenhancing central area. The nodule was shown to be fluorine 18 (18F) fluorodeoxyglucose (FDG)–avid on PET/CT scan (Fig 1C) (maximum standardized uptake value, 9.3). There were no other findings or suspected metastatic lesions at PET/CT. The lesion was diagnosed as an intrapericardial paraganglioma at histologic assessment after surgical resection (Fig 2). His blood and urine test results did not reveal alterations (nonfunctioning paraganglioma).Figure 1: Images in a 56-year-old asymptomatic man referred for an elective coronary calcium score stratification. (A, B) Axial CT scans, (C) axial two-dimensional fluorine 18 (18F) fluorodeoxyglucose (FDG) PET/CT scan, and (D, E) tridimensional reconstructions show a low-attenuation nodule (arrow), measuring 2.6 cm, with intense and heterogeneous postcontrast enhancement, central nonenhancing areas, and 18F-FDG uptake (maximum standardized uptake value, 9.3) surrounded by the inferior border of aortic arch, the posterior border of ascending aorta, the upper border of the right main pulmonary artery, and the anterior border of the trachea and proximal left main bronchus. Note the paraganglioma relationship with the posterior portion of the superior aortic recess (arrowheads).Figure 1:Download as PowerPointOpen in Image Viewer Figure 2: Intrapericardial paraganglioma. (A) Photomicrograph shows encapsulated paraganglioma with tumor cells arranged in solid nests with sustentacular cells, vascular stroma, and abundant eosinophilic cytoplasm, without mitotic figures, involvement of intracapsular vessel, or areas of necrosis. (Hematoxylin-eosin stain; original magnification, ×100; inset original magnification, ×400.) (B) The tumor is diffusely positive for chromogranin-A. (Chromogranin-A immunohistochemical staining; original magnification, ×250.) (C) The sustentacular cells are prominent with S100 staining. (Original magnification, ×400.)Figure 2:Download as PowerPointOpen in Image Viewer Although the clusters of chromaffin cells of the sympathetic ganglia are widely distributed throughout the whole body, cardiac paragangliomas are exceptionally rare (1–3). They can originate from the visceral autonomic (atrium or interatrial septum) or branchiomeric (coronary, pulmonary, and aorticopulmonary) paraganglia, being more commonly located on the roof of the left atrium (3). Furthermore, paragangliomas usually appear as large and circumscribed masses, echogenic at echocardiography, hypoattenuating with intense and heterogeneous postcontrast enhancement at CT, and markedly hyperintense on T2-weighted MR images and iso- or hypointense relative to myocardium on T1-weighted MR images (2).Functional imaging agents are able to target paragangliomas through different mechanisms, mainly through the catecholamine synthesis, storage, and secretion pathways, in addition to associated genetic mutations, with gallium 68 tetraazacyclododecane tetraacetic acid–octreotate (DOTATATE) PET/CT being significantly superior when compared with 18F-FDG, 18F-fluorodopamine (FDA), and 18F-fluorodihydroxyphenylalanine (FDOPA) and in comparison to CT and MRI (4,5). Despite being a sensitive but nonspecific radiopharmaceutical that enters the cell via glucose transporters, the presence of active brown adipose tissue on 18F-FDG PET/CT scans is associated with higher mortality and cancer activity, once its activation is mediated through the action of norepinephrine on β-adrenoceptors when secreted by pheochromocytomas or paragangliomas and in some malignancies (4,6).Disclosures of conflicts of interest: L.d.P.G.d.F. No relevant relationships. G.B.d.S.T. No relevant relationships. L.d.P.S.B. No relevant relationships. A.S.d.A. No relevant relationships.Keywords: CT Angiography, Image Postprocessing, PET/CT, Cardiac, Pericardium, Heart, NeoplasmsAuthors declared no funding for this work.References1. Hamilton BH , Francis IR , Gross BH , et al. Intrapericardial paragangliomas (pheochromocytomas): imaging features. AJR Am J Roentgenol 1997;168(1):109–113. Crossref, Medline, Google Scholar2. Araoz PA , Mulvagh SL , Tazelaar HD , Julsrud PR , Breen JF . 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Crossref, Google ScholarArticle HistoryReceived: May 17 2022Revision requested: June 1 2022Revision received: June 13 2022Accepted: June 27 2022Published online: July 28 2022 FiguresReferencesRelatedDetailsRecommended Articles Anterior Subtype of Partial Anomalous Left Pulmonary ArteryRadiology: Cardiothoracic Imaging2023Volume: 5Issue: 2Congenital Variants and Anomalies of the Aortic ArchRadioGraphics2016Volume: 37Issue: 1pp. 32-51Tracheobronchial Branching Abnormalities: Lobe-based Classification SchemeRadioGraphics2016Volume: 36Issue: 2pp. 358-373Role of CT in the Pre- and Postoperative Assessment of Conotruncal AnomaliesRadiology: Cardiothoracic Imaging2022Volume: 4Issue: 3Side Effects of Oncologic Treatment in the Chest: Manifestations at FDG PET/CTRadioGraphics2021Volume: 41Issue: 7pp. 2071-2089See More RSNA Education Exhibits Pericardial Recesses on Multidetector CT: Beyond Tips and Tricks Around the HeartDigital Posters2022Typical and Atypical Pulmonary Artery Slings and PALPA: Clinical and Imaging ManifestationsDigital Posters2020Advanced Cardiovascular Computed Tomography Imaging in Children with Congenital Variants and Anomalies of the Aortic Arch: From Volume Rendering to 3D Models and 3D PrintingDigital Posters2022 RSNA Case Collection Aortopulmonary fistula due to aortic dissection RSNA Case Collection2020Tracheal bronchusRSNA Case Collection2021Aberrant left subclavian artery from right-sided aortic archRSNA Case Collection2020 Vol. 4, No. 4 Metrics Altmetric Score PDF download