Skip to main content

A Clinico-Pathologic Approach to the Differential Diagnosis of Pericardial Tumors

Abstract

Purpose of Review

Tumors of the pericardium are rare, but a wide variety of congenital, infectious, inflammatory, and neoplastic processes have been reported. Pericardial tumors can be categorized as non-neoplastic or neoplastic. Neoplastic lesions can be further divided into benign or malignant, with malignancies being either primary or secondary (metastatic). Clinical, radiographic, and pathologic features of the most common entities are discussed.

Recent Findings

Metastatic neoplasms involving the heart and pericardium are far more common than primary pericardial neoplasms. Of primary pericardial malignancies, mesothelioma is the most common; notably, cytology of effusion fluid is relatively insensitive to the diagnosis. The prognosis for most malignancies of the pericardium, primary or secondary, is poor. Increasingly, clinically recognized diseases that involve the pericardium include Erdheim-Chester and IgG4-related disease.

Summary

This article provides a comprehensive review of the most recent literature to develop a structured framework to the differential diagnosis of pericardial tumors.

This is a preview of subscription content, access via your institution.

Fig. 1

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Rodriguez ER, Tan CD. Structure and anatomy of the human pericardium. Prog Cardiovasc Dis. 2017;59(4):327–40.

    PubMed  Article  Google Scholar 

  2. 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(1):69–75.

    PubMed  Article  Google Scholar 

  3. 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):1027–31.

    CAS  PubMed  Google Scholar 

  4. Grebenc ML, et al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000;20(4):1073–103 quiz 1110-1, 1112.

    CAS  PubMed  Article  Google Scholar 

  5. Restrepo CS, Vargas D, Ocazionez D, Martínez-Jiménez S, Betancourt Cuellar SL, Gutierrez FR. Primary pericardial tumors. Radiographics. 2013;33(6):1613–30.

    PubMed  Article  Google Scholar 

  6. Garcia-Riego A, Cuinas C, Vilanova JJ. Malignant pericardial effusion. Acta Cytol. 2001;45(4):561–6.

    CAS  PubMed  Article  Google Scholar 

  7. Burke, A., Tumors of the heart and great vessels (Atlas of Tumor Pathology). Atlas of Tumor Pathology - Series 4, ed. S.G. Silverberg. Vol. 22. 2015, Silver Spring, MD, USA: American Registry of Pathology

  8. Alkharabsheh S, et al. Clinical features, natural history, and management of pericardial cysts. Am J Cardiol. 2019;123(1):159–63 This article describes a retrospective analysis of 103 patients with pericardial cysts to determine the natural course of disease.

    PubMed  Article  Google Scholar 

  9. Khayata M, Alkharabsheh S, Shah NP, Klein AL. Pericardial cysts: a contemporary comprehensive review. Curr Cardiol Rep. 2019;21(7):64.

    PubMed  Article  Google Scholar 

  10. Tower-Rader A, Kwon D. Pericardial masses, cysts and diverticula: a comprehensive review using multimodality imaging. Prog Cardiovasc Dis. 2017;59(4):389–97 This article provides a thorough review of the characteristics of pericardial tumors, cysts, and diverticula by echocardiography, computed tomography, and magnetic resonance imaging.

    PubMed  Article  Google Scholar 

  11. Parmar YJ, Shah AB, Poon M, Kronzon I. Congenital abnormalities of the pericardium. Cardiol Clin. 2017;35(4):601–14.

    PubMed  Article  Google Scholar 

  12. Najib MQ, Chaliki HP, Raizada A, Ganji JL, Panse PM, Click RL. Symptomatic pericardial cyst: a case series. Eur J Echocardiogr. 2011;12(11):E43.

    PubMed  Google Scholar 

  13. Davis M, Carneiro A, Dymarkowski S, Bogaert J. Bronchogenic cyst masquerading as pericarditis. Circulation. 2010;122(7):e426–8.

    PubMed  Article  Google Scholar 

  14. Ramireddy K, et al. Bronchogenic cyst presenting as acute pericarditis. Cureus. 2020;12(6):e8874.

    PubMed  PubMed Central  Google Scholar 

  15. Al-Ani M, et al. Pericardial mass in a patient with rheumatoid arthritis. BMJ Case Rep. 2015;2015.

  16. Canas F, et al. A giant fibrinoid pericardial mass in a patient with rheumatoid arthritis: a case report. Eur Heart J Case Rep. 2019;3(2).

  17. Okajima K, Posas-Mendoza T, Tran DD, Hong RA. Rheumatoid arthritis and cardiac compression caused by a large fibrotic intrapericardial mass and effusion: a case report. Am J Case Rep. 2019;20:1120–3.

    PubMed  PubMed Central  Article  Google Scholar 

  18. Bhat P, Chandrasekaran B, Barnes E. Cardiac compression from a rheumatoid pericardial mass. Heart. 2006;92(9):1337.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  19. Mori K, Yamada K, Konno T, Inoue D, Uno Y, Watanabe M, et al. Pericardial involvement in IgG4-related disease. Intern Med. 2015;54(10):1231–5.

    PubMed  Article  Google Scholar 

  20. Oyama-Manabe N, et al. IgG4-related cardiovascular disease from the aorta to the coronary arteries: multidetector CT and PET/CT. Radiographics. 2018;38(7):1934–48 This article describes the most common locations affected by IgG4-RD in the cardiovascular system, and the approach to diagnosis by imaging.

    PubMed  Article  Google Scholar 

  21. Yassi U, Iqbal F, Stevenson HL. IgG4-related sclerosing pericarditis in a young man with recurrent chest pain. Ann Thorac Surg. 2019;108(4):e261–3.

    PubMed  Article  Google Scholar 

  22. Deshpande V, Zen Y, Chan JKC, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.

    PubMed  Article  Google Scholar 

  23. Frydman J, Grunner S, Kluger Y. IgG4-related disease manifesting as an acute gastric-pericardial fistula. World J Gastroenterol. 2014;20(44):16782–5.

    PubMed  PubMed Central  Article  Google Scholar 

  24. Luthringer DJ, Virmani R, Weiss SW, Rosai J. A distinctive cardiovascular lesion resembling histiocytoid (epithelioid) hemangioma. Evidence suggesting mesothelial participation. Am J Surg Pathol. 1990;14(11):993–1000.

    CAS  PubMed  Article  Google Scholar 

  25. Pham TT, Antons K, Shishido R, Mullvain J, Salem F, Haghighi P. A case of mesothelial/monocytic cardiac excrescence causing severe acute cardiopulmonary failure. Am J Surg Pathol. 2005;29(4):564–7.

    PubMed  Article  Google Scholar 

  26. Argani P, Sternberg S, Burt M, Adsay N, Klimstra D. Metastatic adenocarcinoma involving a mesothelial/monocytic incidental cardiac excrescence (cardiac MICE). Am J Surg Pathol. 1997;21(8):970–4.

    CAS  PubMed  Article  Google Scholar 

  27. Courtice RW, Stinson WA, Walley VM. Tissue fragments recovered at cardiac surgery masquerading as tumoral proliferations. Evidence suggesting iatrogenic or artefactual origin and common occurrence. Am J Surg Pathol. 1994;18(2):167–74.

    CAS  PubMed  Article  Google Scholar 

  28. Pruente R, Restrepo CS, Ocazionez D, Suby-Long T, Vargas D. Fatty lesions in and around the heart: a pictorial review. Br J Radiol. 2015;88(1051):20150157.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  29. Franco-Paredes C, Rouphael N, Méndez J, Folch E, Rodríguez-Morales AJ, Santos JI, et al. Cardiac manifestations of parasitic infections part 3: pericardial and miscellaneous cardiopulmonary manifestations. Clin Cardiol. 2007;30(6):277–80.

    PubMed  PubMed Central  Article  Google Scholar 

  30. Gulati GS, Sharma S. Pericardial abscess occurring after tuberculous pericarditis: image morphology on computed tomography and magnetic resonance imaging. Clin Radiol. 2004;59(6):514–9.

    CAS  PubMed  Article  Google Scholar 

  31. Zhou W, Srichai MB. Multi-modality imaging assessment of pericardial masses. Curr Cardiol Rep. 2017;19(4):32.

    PubMed  Article  Google Scholar 

  32. Durak D, Eren B. Pericardial lipoma: an autopsy case and review of the literature. J Forensic Sci. 2007;52(4):949–50.

    PubMed  Article  Google Scholar 

  33. Avondo S, et al. Update on diagnosis and management of neoplastic pericardial disease. Expert Rev Cardiovasc Ther. 2020;18(9):615–23 This article reviews the radiographic and pathologic diagnosis, and surgical and medical management, of pericardial neoplasms.

    CAS  PubMed  Article  Google Scholar 

  34. Husain M, McCanta A, Batra AS. Rare finding of a pericardial lipoma in a pediatric patient presenting with ventricular tachycardia. HeartRhythm Case Rep. 2019 Feb 23;5(5):277–80.

    PubMed  PubMed Central  Article  Google Scholar 

  35. Ko SF, Shieh CS, Shih TY, Hsiao CC, Ng SH, Lee TY, et al. Mediastinal lipoblastoma with intraspinal extension: MRI demonstration. Magn Reson Imaging. 1998;16(4):445–8.

    CAS  PubMed  Article  Google Scholar 

  36. Geramizadeh B, Javadi F, Foroutan HR. Intrathoracic lipoblastoma in a 15-month-old infant. Rare Tumors. 2011;3(4):e51.

    PubMed  PubMed Central  Google Scholar 

  37. Burke A, Tavora F. The 2015 WHO classification of tumors of the heart and pericardium. J Thorac Oncol. 2016;11(4):441–52.

    PubMed  Article  Google Scholar 

  38. MacKenzie S, Loken S, Kalia N, Trevenen C, Harder J, Wong A, et al. Intrapericardial teratoma in the perinatal period. Case report and review of the literature. J Pediatr Surg. 2005;40(12):e13–8.

    PubMed  Article  Google Scholar 

  39. Bader R, Hornberger LK, Nijmeh LJ, al-Kazaleh F, Ryan G, Toi A, et al. Fetal pericardial teratoma: presentation of two cases and review of literature. Am J Perinatol. 2006;23(1):53–8.

    PubMed  Article  Google Scholar 

  40. Burke A, Virmani R. Pediatric heart tumors. Cardiovasc Pathol. 2008;17(4):193–8.

    PubMed  Article  Google Scholar 

  41. Luk A, Ahn E, Vaideeswar P, Butany JW. Pericardial tumors. Semin Diagn Pathol. 2008;25(1):47–53.

    PubMed  Article  Google Scholar 

  42. Almobarak AA, AlShammari A, Alhomoudi RI, Eshaq AM, Algain SM, Jensen EC, et al. Benign pericardial schwannoma: case report and summary of previously reported cases. Am J Case Rep. 2018;19:90–4.

    PubMed  PubMed Central  Article  Google Scholar 

  43. Lamba G, Frishman WH. Cardiac and pericardial tumors. Cardiol Rev. 2012;20(5):237–52.

    PubMed  Article  Google Scholar 

  44. Siejka DA, et al. Resection of a functioning intrapericardial paraganglioma associated with succinate dehydrogenase B mutation. SAGE Open Med Case Rep. 2019;7:2050313X19839530.

    PubMed  PubMed Central  Google Scholar 

  45. Chen YY, Huang WC, Huang MH, Lu TM, Hsu CP. The intra-pericardial paraganglioma presenting as ascites and hemopericardium with impending tamponade. Int Heart J. 2018;59(3):664–7.

    PubMed  Article  Google Scholar 

  46. Ojiaku M, Peña E, Belanger EC, Chan KL, Dennie C. Functioning intrapericardial paraganglioma: multimodality imaging findings and pathological correlation. Circulation. 2014;130(16):e137–9.

    PubMed  Article  Google Scholar 

  47. Rotolo N, et al. Management of hypertension in intrapericardial paraganglioma. Int J Hypertens. 2014;2014:812598.

    PubMed  PubMed Central  Article  CAS  Google Scholar 

  48. Sanchez-Nadales A, Anampa-Guzman A, Navarro-Motta J. Erdheim-Chester disease with extensive pericardial involvement: a case report and systematic review. Cardiol Res. 2020;11(2):118–28.

    PubMed  PubMed Central  Article  Google Scholar 

  49. Costa I, Abdo ANR, Bittar CS, Fonseca SMR, Moraes ASHT, Kalil Filho R, et al. Cardiovascular manifestations of Erdheim-Chester's disease: a case series. Arq Bras Cardiol. 2018;111(6):852–5.

    PubMed  PubMed Central  Google Scholar 

  50. Chahine J, Alzubi J, Alnajjar H, Ramchand J, Chetrit M, Klein AL. Erdheim-Chester disease: a rare but important cause of recurrent pericarditis. Curr Cardiol Rep. 2020;22(8):75.

    PubMed  Article  Google Scholar 

  51. Ghotra AS, et al. Cardiovascular manifestations of Erdheim-Chester disease. Echocardiography. 2019;36(2):229–36 This article is a review of the cardiovascular manifestations of Erdheim-Chester disease in 24 biopsy-proved affected patients.

    PubMed  Article  Google Scholar 

  52. Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, et al. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore). 2004;83(6):371–92.

    Article  Google Scholar 

  53. Haroche J, Cluzel P, Toledano D, Montalescot G, Touitou D, Grenier PA, et al. Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 2009;119(25):e597–8.

    PubMed  Article  Google Scholar 

  54. Czimbalmos C, Csecs I, Polos M, Bartha E, Szucs N, Toth A, et al. Uncommon presentation of a rare tumour - incidental finding in an asymptomatic patient: case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours. BMC Cancer. 2017;17(1):612.

    PubMed  PubMed Central  Article  Google Scholar 

  55. Zhang LP, Zhang L, Wang G, Adhikari BK, Liu Q, Zhang W. Pericardial malignant solitary fibrous tumour with right atrial invasion - a case report and literature review. J Int Med Res. 2019;47(6):2716–22.

    PubMed  PubMed Central  Article  Google Scholar 

  56. Taguchi S. Primary cardiac solitary fibrous tumors. Ann Thorac Cardiovasc Surg. 2015;21(4):329–31.

    PubMed  PubMed Central  Article  Google Scholar 

  57. Antman K, et al. Update on malignant mesothelioma. Oncology (Williston Park). 2005;19(10):1301–9 discussion 1309-10, 1313-6.

    Google Scholar 

  58. Thomason R, Schlegel W, Lucca M, Cummings S, Lee S. Primary malignant mesothelioma of the pericardium. Case report and literature review. Tex Heart Inst J. 1994;21(2):170–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  59. Patel J, Sheppard MN. Primary malignant mesothelioma of the pericardium. Cardiovasc Pathol. 2011;20(2):107–9.

    PubMed  Article  Google Scholar 

  60. Bendek M, Ferenc M, Freudenberg N. Post-irradiation pericardial malignant mesothelioma: an autopsy case and review of the literature. Cardiovasc Pathol. 2010;19(6):377–9.

    PubMed  Article  Google Scholar 

  61. McGehee E, et al. Treatment and outcomes of primary pericardial mesothelioma: a contemporary review of 103 published cases. Clin Lung Cancer. 2019;20(2):e152–7 This article provides aggregated data regarding primary pericardial mesothelioma demographics, clinical presentations, exposure history, treatment, and outcome in 103 published cases.

    PubMed  Article  Google Scholar 

  62. Antman K, Shemin R, Ryan L, Klegar K, Osteen R, Herman T, et al. Malignant mesothelioma: prognostic variables in a registry of 180 patients, the Dana-Farber Cancer Institute and Brigham and Women's Hospital experience over two decades, 1965-1985. J Clin Oncol. 1988;6(1):147–53.

    CAS  PubMed  Article  Google Scholar 

  63. Maleszewski JJ, Anavekar NS. Neoplastic pericardial disease. Cardiol Clin. 2017;35(4):589–600 This article provides a review of the pathologic and radiologic characteristics of the more common pericardial neoplasms.

    PubMed  Article  Google Scholar 

  64. Duran-Moreno J, et al. Pericardial synovial sarcoma: case report, literature review and pooled analysis. In Vivo. 2019;33(5):1531–8.

    PubMed  PubMed Central  Article  Google Scholar 

  65. Wong KY, Hong E, Fong CM, Wong PS. Pericardial synovial sarcoma presenting with unstable angina. Asian Cardiovasc Thorac Ann. 2020;28(1):59–61.

    PubMed  Article  Google Scholar 

  66. Goldblatt J, Saxena P, McGiffin DC, Zimmet A. Pericardial synovial sarcoma: a rare clinical entity. J Card Surg. 2015;30(11):801–4.

    PubMed  Article  Google Scholar 

  67. Ma GT, Liu JZ, Miao Q, Cui QC. Angiosarcoma of the pericardium: a case report. Int J Clin Exp Pathol. 2015;8(10):13568–70.

    PubMed  PubMed Central  Google Scholar 

  68. Killion MJ, Brodovsky HS, Schwarting R. Pericardial angiosarcoma after mediastinal irradiation for seminoma. A case report and a review of the literature. Cancer. 1996;78(4):912–7.

    CAS  PubMed  Article  Google Scholar 

  69. Randhawa JS, Budd GT, Randhawa M, Ahluwalia M, Jia X, Daw H, et al. Primary cardiac sarcoma: 25-year Cleveland clinic experience. Am J Clin Oncol. 2016;39(6):593–9.

    PubMed  Article  Google Scholar 

  70. Lestuzzi C, Berretta M, Tomkowski W. 2015 update on the diagnosis and management of neoplastic pericardial disease. Expert Rev Cardiovasc Ther. 2015;13(4):377–89.

    CAS  PubMed  Article  Google Scholar 

  71. Jeudy J, Burke AP, Frazier AA. Cardiac lymphoma. Radiol Clin N Am. 2016;54(4):689–710.

    PubMed  Article  Google Scholar 

  72. Patel S, Xiao P. Primary effusion lymphoma. Arch Pathol Lab Med. 2013;137(8):1152–4.

    PubMed  Article  Google Scholar 

  73. Chintala K, Bloom DA, Walters HL 3rd, Pettersen MD. Images in cardiology: pericardial yolk sac tumor presenting as cardiac tamponade in a 21-month-old child. Clin Cardiol. 2004;27(7):411.

    PubMed  Article  Google Scholar 

  74. Shi L, Wu L, Fang H, Han B, Yang J, Ma X, et al. Identification and clinical course of 166 pediatric cardiac tumors. Eur J Pediatr. 2017;176(2):253–60.

    PubMed  Article  Google Scholar 

  75. Bussani R, de-Giorgio F, Abbate A, Silvestri F. Cardiac metastases. J Clin Pathol. 2007;60(1):27–34.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  76. Dermawan JKT, Policarpio-Nicolas ML. Malignancies in pleural, peritoneal, and pericardial effusions. Arch Pathol Lab Med. 2020;144(9):1086–91 This document provides a recent review of the incidence and type of malignancy in 155 malignant pericardial effusions.

    PubMed  Article  Google Scholar 

  77. He B, Yang Z, Zhao P, Li YJ, Wang JG. Cytopathologic analysis of pericardial effusions in 116 cases: implications for poor prognosis in lung cancer patients with positive interpretations. Diagn Cytopathol. 2017;45(4):287–93.

    PubMed  Article  Google Scholar 

  78. Dragoescu EA, Liu L. Pericardial fluid cytology: an analysis of 128 specimens over a 6-year period. Cancer Cytopathol. 2013;121(5):242–51.

    CAS  PubMed  Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Alison R. Krywanczyk.

Ethics declarations

Conflict of Interest

The authors have no conflicts of interest to report.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Pericardial Disease

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Krywanczyk, A.R., Tan, C.D. & Rodriguez, E.R. A Clinico-Pathologic Approach to the Differential Diagnosis of Pericardial Tumors. Curr Cardiol Rep 23, 119 (2021). https://doi.org/10.1007/s11886-021-01548-6

Download citation

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s11886-021-01548-6

Keywords

  • Pericardium
  • Pericardial mass
  • Pericardial tumor
  • Pericardium metastasis
  • Pericardial effusion