CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2023; 33(03): 394-399
DOI: 10.1055/s-0041-1741092
Case Series

Multimodality Imaging Appearance of Intrapericardial Paragangliomas

Diana Palacio
Maria del Pilar Gutierrez
2   Department of Radiology, Universidad de Antioquia-Facultad de Medicina, Tucson, Arizona, United States
Gokhan Kuyumcu
1   Department of Medical Imaging, The University of Arizona College of Medicine, Tucson, Arizona, United States
Benjamin Rounseville
1   Department of Medical Imaging, The University of Arizona College of Medicine, Tucson, Arizona, United States
Volodymyr Shponka
3   Department of Pathology, The University of Arizona College of Medicine, Tucson, Arizona, United States
Sonia Betancourt
4   Department of Radiology, The University of Texas MD Anderson Cancer Center, Tucson, Arizona, United States
› Author Affiliations


Paragangliomas are neuroendocrine tumors of the sympathetic and parasympathetic nervous system that originate from neural crest cells. Less than 1% of paragangliomas are found in the heart, originating from intrinsic cardiac ganglia cells in the posterior wall the atria, atrioventricular groove, and along the root of the great vessels. A 10-year review of our institution's database identified nine patients who had documented intrapericardial paragangliomas. We describe the multimodality imaging appearance of these tumors. The most common findings include embedment and wrapping around the great vessels and atrioventricular groove within the confines of the pericardium, markedly avid heterogeneous enhancement, distinct engorged neovascularization, and in large lesions, central low attenuation areas compatible with hemorrhage, necrosis, or cystic degeneration.

Supplementary Material

Publication History

Article published online:
31 January 2023

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  • References

  • 1 Aravot DJ, Banner NR, Cantor AM, Theodoropoulos S, Yacoub MH. Location, localization and surgical treatment of cardiac pheochromocytoma. Am J Cardiol 1992; 69 (03) 283-285
  • 2 Brown ML, Zayas GE, Abel MD, Young Jr WF, Schaff HV. Mediastinal paragangliomas: the mayo clinic experience. Ann Thorac Surg 2008; 86 (03) 946-951
  • 3 Hamilton BH, Francis IR, Gross BH. et al. Intrapericardial paragangliomas (pheochromocytomas): imaging features. Am J Roentgenol 1997; 168 (01) 109-113
  • 4 Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993; 117 (10) 1027-1031
  • 5 Ramlawi B, David EA, Kim MP. et al. Contemporary surgical management of cardiac paragangliomas. Ann Thorac Surg 2012; 93 (06) 1972-1976
  • 6 DeLellis RALR, Heitz PU. et al. Pathology and Genetics of Tumours of Endocrine Organs. Third edition.. Lyon France: 2004
  • 7 Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K. North American Neuroendocrine Tumor Society (NANETS). The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 2010; 39 (06) 775-783
  • 8 Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer 2014; 38 (01) 7-41
  • 9 Tischler AS. Pheochromocytoma and extra-adrenal paraganglioma: updates. Arch Pathol Lab Med 2008; 132 (08) 1272-1284
  • 10 Ayala-Ramirez M, Feng L, Johnson MM. et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab 2011; 96 (03) 717-725
  • 11 Plouin PF, Fitzgerald P, Rich T. et al. Metastatic pheochromocytoma and paraganglioma: focus on therapeutics. Horm Metab Res 2012; 44 (05) 390-399
  • 12 Khan MF, Datta S, Chisti MM, Movahed MR. Cardiac paraganglioma: clinical presentation, diagnostic approach and factors affecting short and long-term outcomes. Int J Cardiol 2013; 166 (02) 315-320
  • 13 Gomi T, Ikeda T, Sakurai J, Toya Y, Tani M. Cardiac pheochromocytoma. A case report and review of the literature. Jpn Heart J 1994; 35 (01) 117-124
  • 14 Jeevanandam V, Oz MC, Shapiro B, Barr ML, Marboe C, Rose EA. Surgical management of cardiac pheochromocytoma. Resection versus transplantation. Ann Surg 1995; 221 (04) 415-419
  • 15 Jönsson A, Hallengren B, Manhem P. et al. Cardiac pheochromocytoma. J Intern Med 1994; 236 (01) 93-96
  • 16 Lewis IH, Yousif D, Mullis SL, Homma S, Gabrielson GV, Jebara VA. Case 2–1994. Management of a cardiac pheochromocytoma in two patients. J Cardiothorac Vasc Anesth 1994; 8 (02) 223-230
  • 17 Paul S, Jain SH, Gallegos RP, Aranki SF, Bueno R. Functional paraganglioma of the middle mediastinum. Ann Thorac Surg 2007; 83 (06) e14-e16
  • 18 Huo JL, Choi JC, DeLuna A. et al. Cardiac paraganglioma: diagnostic and surgical challenges. J Card Surg 2012; 27 (02) 178-182
  • 19 Agarwal PP, Seely JM, Matzinger FR. Case 130: mediastinal hemangioma. Radiology 2008; 246 (02) 634-637
  • 20 Meng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol 2002; 84 (01) 69-75
  • 21 Goldberg HP, Steinberg I. Primary tumors of the heart. Circulation 1955; 11 (06) 963-970
  • 22 Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 2000; 20 (04) 1073-1103 , quiz 1110–1111, 1112
  • 23 Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: the great mimic. Radiographics 2011; 31 (06) 1793-1807
  • 24 Patnana M, Sevrukov AB, Elsayes KM, Viswanathan C, Lubner M, Menias CO. Inflammatory pseudotumor: the great mimicker. Am J Roentgenol 2012; 198 (03) W217-227