Skip to main content

Cardiac Tumours

  • Chapter
  • First Online:
Cardiac Pathology

Abstract

Cardiac tumours are rare. The majority are metastatic from elsewhere, with primary cardiac tumours representing only 0.02% of tumours in autopsy series. Their prevalence in the general population is difficult to estimate. Malignancies associated with metastasis to the heart include melanoma, carcinomas (most commonly of the lung and breast), sarcomas and lymphomas.

The clinical presentation of cardiac tumours is varied and is usually dependent upon the site and size of the lesion, rate of growth and invasiveness, and any associated complications such as pericardial effusion, conduction disturbances, cardiac failure and systemic or pulmonary emboli. Non-specific constitutional symptoms such as fever, malaise and weight loss may also be encountered.

With the exception of lymphoma, the treatment of choice for cardiac tumours is complete resection in combination with chemotherapy or radiotherapy, although targeted therapies may provide future therapeutic options. Some cardiac tumours are part of genetic syndromes or are associated with a genetic predisposition and therefore have implications for genetic counselling.

Over 75–90% of primary cardiac tumours are benign, and the prevalence of each histological type of primary cardiac tumour is age-related. Cardiac myxomas are the most common benign cardiac neoplasms, although due to better imaging techniques papillary fibroelastomas may be more frequent than previously thought. Other benign lesions of the heart include rhabdomyoma, cardiac fibroma, cystic tumour of the atrioventricular node, cardiac lipoma and haemangioma. The heart may rarely be involved by inflammatory myofibroblastic tumour. Other primary cardiac entities include histiocytoid cardiomyopathy and lipomatous hypertrophy.

Most malignant cardiac tumours are sarcomas, comprising rhabdomyosarcoma, which predominates in children, angiosarcoma (representing 40% of cardiac sarcomas) and other types of sarcoma. Up to 2% of malignant cardiac lesions represent non-Hodgkin lymphoma.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Yu K, et al. Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact Cardiovasc Thorac Surg. 2007;6(5):636–9.

    Article  PubMed  Google Scholar 

  2. Hoffmeier A, et al. Cardiac tumors--diagnosis and surgical treatment. Dtsch Arztebl Int. 2014;111(12):205–11.

    Google Scholar 

  3. Tazelaar HD, Maleszewski JJ. Tumours of the heart and pericardium. In: Fletcher CDM, editor. Diagnostic histopathology of tumours. Philadelphia, PA: Elsevier Saunders; 2013. p. 6–41.

    Google Scholar 

  4. Burazor I, et al. Metastatic cardiac tumors: from clinical presentation through diagnosis to treatment. BMC Cancer. 2018;18(1):202.

    Google Scholar 

  5. Sheppard MN. Practical cardiovascular pathology. 2nd ed. Boca Raton, FL: CRC Press; 2011.

    Google Scholar 

  6. Suvarna SK, Royds JA. The nature of the cardiac myxoma. Int J Cardiol. 1996;57(3):211–6.

    Article  CAS  PubMed  Google Scholar 

  7. Krikler DM, et al. Atrial myxoma: a tumour in search of its origins. Br Heart J. 1992;67(1):89–91.

    Article  CAS  Google Scholar 

  8. Jain D, Maleszewski JJ. Cardiac myxoma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: International Agency for Research on Cancer Press; 2015.

    Google Scholar 

  9. Thomas-de-Montpréville V, et al. Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center. Cardiovasc Pathol. 2007;16(1):22–8.

    Article  PubMed  Google Scholar 

  10. Bjessmo S, Ivert T. Cardiac myxoma: 40 years’ experience in 63 patients. Ann Thorac Surg. 1997;63(3):697–700.

    Article  CAS  PubMed  Google Scholar 

  11. Pucci A, et al. Histopathologic and clinical characterization of cardiac myxoma: review of 53 cases from a single institution. Am Heart J. 2000;140(1):134–8.

    Article  CAS  PubMed  Google Scholar 

  12. Reynen K. Cardiac myxomas. N Engl J Med. 2018;333(24):1610–7.

    Article  CAS  PubMed  Google Scholar 

  13. Di Vito A, Mignogna C, Donato G. The mysterious pathways of cardiac myxomas: a review of histogenesis, pathogenesis and pathology. Histopathology. 2015;66(3):321–32.

    Google Scholar 

  14. McCarthy PM, et al. The significance of multiple, recurrent, and “complex” cardiac myxomas. J Thorac Cardiovasc Surg. 1986;91(3):389–96.

    Google Scholar 

  15. Edwards A, et al. Carney’s syndrome: complex myxomas. Report of four cases and review of the literature. Cardiovasc Surg. 2002;10(3):264–75.

    Article  CAS  PubMed  Google Scholar 

  16. Sheppard M. Cardiac myxomas. In: Practical cardiovascular pathology. London: Arnold; 2011.

    Chapter  Google Scholar 

  17. Keeling IM, et al. Cardiac myxomas: 24 years of experience in 49 patients. Eur J Cardiothorac Surg. 2002;22(6):971–7.

    Article  CAS  PubMed  Google Scholar 

  18. Burke A, Virmani R. More on cardiac myxomas. N Engl J Med. 1996;335(19):1462–3; author reply 1463–4.

    Google Scholar 

  19. Kusumi T, et al. Cardiac tumor comprising two components including typical myxoma and atypical hypercellularity suggesting a malignant change. Cardiovasc Pathol. 2009;18(6):369–74.

    Article  PubMed  Google Scholar 

  20. Chin RI, et al. Papillary fibroelastoma as a cause of cardiogenic embolic stroke in a β-thalassemia patient: case report and literature review. Case Rep Cardiol. 2017;2017:8185601.

    Article  Google Scholar 

  21. Fleischmann KE, Schiller NB. Papillary fibroelastoma: move over myxoma. J Am Coll Cardiol. 2015;65(22):2430–2.

    Article  PubMed  Google Scholar 

  22. Mariscalco G, et al. Papillary fibroelastoma: insight to a primary cardiac valve tumor. J Card Surg. 2010;25(2):198–205.

    Article  PubMed  Google Scholar 

  23. Maleszewski JJ, Agaimy A. Papillary fibroelastoma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  24. Hakim FA, et al. Papillary fibroelastoma of the pulmonary valve--a systematic review. Echocardiography. 2014;31(2):234–40.

    Article  PubMed  Google Scholar 

  25. Sheppard M. Papillary fibroelastoma. In: Practical cardiovascular pathology. London: Arnold; 2011.

    Chapter  Google Scholar 

  26. Kumar TK, et al. Multiple papillary fibroelastomas of the heart. Ann Thorac Surg. 2009;88(6):e66–7.

    Article  PubMed  Google Scholar 

  27. Sydow K, et al. Papillary fibroelastomas of the heart. Thorac Cardiovasc Surg. 2008;56(1):9–13.

    Article  CAS  PubMed  Google Scholar 

  28. Akay MH, Seiffert M, Ott DA. Papillary fibroelastoma of the aortic valve as a cause of transient ischemic attack. Tex Heart Inst J. 2009;36(2):158–9.

    Google Scholar 

  29. Hirose H, et al. Left atrial fibroelastoma. Ann Thorac Cardiovasc Surg. 2009;15(6):412–4.

    Google Scholar 

  30. Ibrahim CPH, et al. Cardiac rhabdomyoma presenting as left ventricular outflow tract obstruction in a neonate. Interact Cardiovasc Thorac Surg. 2018;2(4):572–4.

    Article  CAS  PubMed  Google Scholar 

  31. Krasuski RA, et al. Cardiac rhabdomyoma in an adult patient presenting with ventricular arrhythmia. Chest. 2000;118(4):1217–21.

    Article  CAS  PubMed  Google Scholar 

  32. Kawada H, et al. Multiple cutaneous rhabdomyomas in a child. Eur J Dermatol. 2004;14(6):418–20.

    Google Scholar 

  33. Chao AS, et al. Outcome of antenatally diagnosed cardiac rhabdomyoma: case series and a meta-analysis. Ultrasound Obstet Gynecol. 2008;31(3):289–95.

    Article  CAS  PubMed  Google Scholar 

  34. Lee E, et al. Primary cardiac tumors associated with genetic syndromes: a comprehensive review. Pediatr Radiol. 2018;48(2):156–64.

    Article  PubMed  Google Scholar 

  35. Amonkar GP, Kandalkar BM, Balasubramanian M. Cardiac rhabdomyoma. Cardiovasc Pathol. 2009;18(5):313–4.

    Article  PubMed  Google Scholar 

  36. Uzun O, et al. Cardiac tumours in children. Orphanet J Rare Dis. 2007;2:11.

    Google Scholar 

  37. Ramírez-Marrero MA, et al. Early complete regression of multiple cardiac tumors suggestive of cardiac rhabdomyomas. Rev Esp Cardiol. 2009;62(6):708–9.

    Article  Google Scholar 

  38. Basso C. Rhabdomyoma. In: Who classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  39. Atik E. Late spontaneous regression of obstructive tumor in the mitral valve. Arq Bras Cardiol. 2009;93(4):e60–2; e48–50.

    Google Scholar 

  40. Hinton RB, et al. Cardiovascular manifestations of tuberous sclerosis complex and summary of the revised diagnostic criteria and surveillance and management recommendations from the International Tuberous Sclerosis Consensus Group. J Am Heart Assoc. 2014;3(6):e001493.

    Google Scholar 

  41. Burke AP. Adult cellular rhabdomyoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 310.

    Google Scholar 

  42. Westhoff CC, et al. Actin isoform expression patterns in adult extracardiac and cardiac rhabdomyomas indicate a different cell of origin. Virchows Arch. 2017;470(3):285–90.

    Article  CAS  PubMed  Google Scholar 

  43. Maleszewski JJ. Hamartoma of mature cardiac myocytes. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IRAC Press; 2015.

    Google Scholar 

  44. Fealey ME, et al. Hamartomas of mature cardiac myocytes: report of 7 new cases and review of literature. Hum Pathol. 2008;39(7):1064–71.

    Article  PubMed  Google Scholar 

  45. Bradshaw SH, et al. Left ventricular mesenchymal hamartoma, a new hamartoma of the heart. Cardiovasc Pathol. 2011;20(5):307–14.

    Article  PubMed  Google Scholar 

  46. Shibuya K, Burke AP. Cardiac fibroma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  47. Nwachukwu H, et al. Cardiac fibroma in adults. Cardiovasc Pathol. 2011;20(4):e146–52.

    Article  PubMed  Google Scholar 

  48. Sheppard M. Fibroma. In: Practical cardiovascular pathology. London: Arnold; 2011.

    Chapter  Google Scholar 

  49. Krane M, et al. Right ventricular fibroma in a 61-year-old man. Thorac Cardiovasc Surg. 2009;57(4):235–7.

    Article  CAS  PubMed  Google Scholar 

  50. Agrawal SKB, Rakhit DJ, Livesey S, Pontefract D, Harden SP. Large intra-cardiac benign fibrous tumour presenting in an adult patient identified using MRI – clinical key. Clin Radiol. 2009;64(6):637–40.

    Article  CAS  PubMed  Google Scholar 

  51. de Montpreville VT, et al. Tumours of the heart - introduction. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 301–4.

    Google Scholar 

  52. Gasparovic H, et al. Left ventricular fibroma mimicking an acute coronary syndrome. Ann Thorac Surg. 2006;82(5):1891–2.

    Article  PubMed  Google Scholar 

  53. Parmley LF, et al. The clinical spectrum of cardiac fibroma with diagnostic and surgical considerations: noninvasive imaging enhances management. Ann Thorac Surg. 1988;45(4):455–65.

    Article  CAS  PubMed  Google Scholar 

  54. Cameselle-Teijeiro J, et al. Cystic tumor of the atrioventricular node of the heart appears to be the heart equivalent of the solid cell nests (ultimobranchial rests) of the thyroid. Am J Clin Pathol. 2018;123(3):369–75.

    Article  Google Scholar 

  55. Evans CA, Suvarna SK. Cystic atrioventricular node tumour: not a mesothelioma. J Clin Pathol. 2005;58(11):1232.

    CAS  PubMed  PubMed Central  Google Scholar 

  56. Veinot JP. Cystic tumour of the atrioventricular node. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  57. Law KB, et al. Cystic tumor of the atrioventricular node: rare antemortem diagnosis. Cardiovasc Pathol. 2012;21(2):120–7.

    Article  PubMed  Google Scholar 

  58. Luc JGY, Phan K, Tchantchaleishvili V. Cystic tumor of the atrioventricular node: a review of the literature. J Thorac Dis. 2017;9(9):3313–8.

    Article  PubMed  PubMed Central  Google Scholar 

  59. Saito S, et al. Successful excision of a cystic tumor of the atrioventricular nodal region. Circ J. 2005;69(10):1293–4.

    Article  PubMed  Google Scholar 

  60. Paniagua JR, et al. Cystic tumour of the atrioventricular nodal region: report of a case successfully treated with surgery. Heart. 2000;83(4):E6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Tran TT, et al. Cardiovascular magnetic resonance diagnosis of cystic tumor of the atrioventricular node. J Cardiovasc Magn Reson. 2009;11:13.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Burke AP, Araoz PA. Cystic tumour of the atrioventricular node. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004. p. 272.

    Google Scholar 

  63. Jiao N, et al. Mesothelial/monocytic incidental cardiac excrescence: a case report and review of literature. Int J Clin Exp Pathol. 2014;7(9):6219–24.

    PubMed  PubMed Central  Google Scholar 

  64. Strecker T, et al. Mesothelial/monocytic incidental cardiac excrescences (cardiac MICE) associated with acute aortic dissection: a study of two cases. Int J Clin Exp Pathol. 2015;8(4):3850–6.

    PubMed  PubMed Central  Google Scholar 

  65. Girolami I, et al. Mesothelial/monocytic incidental cardiac excrescences (MICE): report of a case and review of literature with focus on pathogenesis. Cardiovasc Pathol. 2018;36:25–9.

    Article  PubMed  Google Scholar 

  66. Ruggiero NJ, et al. Myocardial perfusion defect caused by intramyocardial lipoma. J Nucl Cardiol. 2008;15(2):286–9.

    Article  PubMed  Google Scholar 

  67. Gulmez O, et al. Lipoma of the right atrium. J Clin Ultrasound. 2009;37(3):185–8.

    Article  PubMed  Google Scholar 

  68. Riva L, et al. Lipomatous hypertrophy of the interatrial septum. Description of a clinical case and literature review. Minerva Cardioangiol. 2006;54(6):789–92.

    CAS  PubMed  Google Scholar 

  69. Sheppard M. Lipomatous hypertrophy of the atrial septum/lipoma. In: Practical cardiovascular pathology. London: Arnold; 2011.

    Chapter  Google Scholar 

  70. Burke AP, Araoz PA. Cardiac lipoma. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IRAC Press; 2004. p. 271.

    Google Scholar 

  71. Arena V, Valerio L, Capelli A. Lipomatous hypertrophy vs. cardiac hibernoma. What criteria differentiate them? Cardiovasc Pathol. 2009;18(4):250–1.

    Article  PubMed  Google Scholar 

  72. Zhang J, et al. Contrasting fatty involvement of the right ventricle: lipoma versus lipomatous hypertrophy. Singapore Med J. 2009;50(10):e342–5.

    CAS  PubMed  Google Scholar 

  73. Dettrick AJ. Lipomatous hypertrophy of the interatrial septum: report of an unusual case. Histopathology. 2009;54(6):777–9.

    Article  PubMed  Google Scholar 

  74. Veinot JP. Lipoma. In: WHO classification of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  75. Sun X, et al. Left ventricular lipoma resected using thoracoscope-assisted limited sternotomy: a case report and literature review. Medicine (Baltimore). 2018;97(31):e11436.

    Article  Google Scholar 

  76. D’Souza J, et al. Invasive cardiac lipoma: a case report and review of literature. BMC Cardiovasc Disord. 2017;17(1):28.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Ganame J, Wright J, Bogaert J. Cardiac lipoma diagnosed by cardiac magnetic resonance imaging. Eur Heart J. 2008;29(6):697.

    Article  PubMed  Google Scholar 

  78. Ucak A, et al. Resection of intrapericardial hibernoma associated with constrictive pericarditis. Interact Cardiovasc Thorac Surg. 2009;9(4):717–9.

    Article  PubMed  Google Scholar 

  79. de Montpreville TV, Maleszewski JJ. Haemangioma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  80. Mongal LS, et al. Enormous right atrial hemangioma in an asymptomatic patient: a case report and literature review. Echocardiography. 2009;26(8):973–6.

    Article  PubMed  Google Scholar 

  81. Eftychiou C, Antoniades L. Cardiac hemangioma in the left ventricle and brief review of the literature. J Cardiovasc Med (Hagerstown). 2009;10(7):565–7.

    Article  Google Scholar 

  82. Yaganti V, et al. Cavernous hemangioma of the mitral valve: a case report and review of literature. J Cardiovasc Med (Hagerstown). 2009;10(5):420–2.

    Article  Google Scholar 

  83. Vargis RS, et al. Pericardial haemangioma: a common tumour in an unusual location: case report and review of literature. J Clin Diagn Res. 2017;11(5):ED15–7.

    PubMed  PubMed Central  Google Scholar 

  84. Brodwater B, et al. Case report. Pericardial hemangioma. J Comput Assist Tomogr. 1996;20(6):954–6.

    Article  CAS  PubMed  Google Scholar 

  85. Kojima S, et al. Cardiac hemangioma: a report of two cases and review of the literature. Heart Vessels. 2003;18(3):153–6.

    Article  PubMed  Google Scholar 

  86. Kasmani R, et al. An incidental right atrial mass: cavernous hemangioma. Am J Med Sci. 2009;338(4):328–9.

    Article  PubMed  Google Scholar 

  87. Abad C, et al. Resection of a left atrial hemangioma. Report of a case and overview of the literature on resected cardiac hemangiomas. Tex Heart Inst J. 2008;35(1):69–72.

    PubMed  PubMed Central  Google Scholar 

  88. Han Y, et al. Cardiac capillary hemangioma: a case report and brief review of the literature. J Clin Ultrasound. 2014;42(1):53–6.

    Article  CAS  PubMed  Google Scholar 

  89. Tazelaar H, et al. Haemangioma. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IRAC Press; 2004. p. 266–7.

    Google Scholar 

  90. Burke AP, et al. Granular cell tumour. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 324.

    Google Scholar 

  91. Burke AP, et al. Schwannoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 325.

    Google Scholar 

  92. Almobarak AA, et al. Benign pericardial schwannoma: case report and summary of previously reported cases. Am J Case Rep. 2018;19:90–4.

    Article  PubMed  PubMed Central  Google Scholar 

  93. Burke AP, et al. Inflammatory myofibroblastic tumour. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  94. Eilers AL, et al. Cardiac inflammatory myofibroblastic tumor: a comprehensive review of the literature. World J Pediatr Congenit Heart Surg. 2014;5(4):556–64.

    Article  PubMed  Google Scholar 

  95. Burke A, et al. Cardiac inflammatory myofibroblastic tumor: a “benign” neoplasm that may result in syncope, myocardial infarction, and sudden death. Am J Surg Pathol. 2007;31(7):1115–22.

    Article  PubMed  Google Scholar 

  96. Di Maria MV, et al. Successful orthotopic heart transplant in an infant with an inflammatory myofibroblastic tumor of the left ventricle. J Heart Lung Transplant. 2008;27(7):792–6.

    Article  PubMed  Google Scholar 

  97. Sebire NJ, et al. Intravascular inflammatory myofibroblastic tumors in infancy. Pediatr Dev Pathol. 2002;5(4):400–4.

    Article  PubMed  Google Scholar 

  98. Pucci A, et al. Asymptomatic inflammatory myofibroblastic tumor of the heart: immunohistochemical profile, differential diagnosis, and review of the literature. Cardiovasc Pathol. 2009;18(3):187–90.

    Article  PubMed  Google Scholar 

  99. Li L, Cerilli LA, Wick MR. Inflammatory pseudotumor (myofibroblastic tumor) of the heart. Ann Diagn Pathol. 2002;6(2):116–21.

    Article  PubMed  Google Scholar 

  100. Miller DV, Tazelaar HD. Cardiovascular pseudoneoplasms. Arch Pathol Lab Med. 2010;134(3):362–8.

    PubMed  Google Scholar 

  101. Shehata BM. Histiocytoid cardiomyopathy. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  102. Sheppard M. Histiocytoid cardiomyopathy. In: Practical cardiovascular pathology. London: Arnold; 2011.

    Chapter  Google Scholar 

  103. Finsterer J. Histiocytoid cardiomyopathy: a mitochondrial disorder. Clin Cardiol. 2008;31(5):225–7.

    Article  PubMed  PubMed Central  Google Scholar 

  104. Prahlow JA, Teot LA. Histiocytoid cardiomyopathy: case report and literature review. J Forensic Sci. 1993;38(6):1427–35.

    CAS  PubMed  Google Scholar 

  105. Planas S, et al. Association of ventricular noncompaction and histiocytoid cardiomyopathy: case report and review of the literature. Pediatr Dev Pathol. 2012;15(5):397–402.

    Article  PubMed  Google Scholar 

  106. Shehata BM, et al. Histiocytoid cardiomyopathy: three new cases and a review of the literature. Pediatr Dev Pathol. 1998;1(1):56–69.

    Article  CAS  PubMed  Google Scholar 

  107. Shehata BM, et al. Benign tumours with myocyte differentiation—histiocytoid cardiomyopathy. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004. p. 256–8.

    Google Scholar 

  108. Rea G, et al. Histiocytoid cardiomyopathy and microphthalmia with linear skin defects syndrome: phenotypes linked by truncating variants in. Cold Spring Harb Mol Case Stud. 2017;3(1):a001271.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  109. Gallo P, d’Amati G. Cardiomyopathies. In: Cardiovascular pathology. London: Churchill Livingstone; 2001.

    Google Scholar 

  110. Burke A. Primary malignant cardiac tumors. Semin Diagn Pathol. 2008;25(1):39–46.

    Article  PubMed  Google Scholar 

  111. Watson R, et al. Primary undifferentiated pleomorphic cardiac sarcoma with. BMJ Case Rep. 2018;2018:bcr-2018-226073.

    Article  PubMed  Google Scholar 

  112. Lestuzzi C. Primary tumors of the heart. Curr Opin Cardiol. 2016;31(6):593–8.

    Article  PubMed  Google Scholar 

  113. Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748–56.

    Article  CAS  Google Scholar 

  114. Burke AP, Virmani R. Tumours of the heart and great vessels, Atlas of tumour pathology. Washington, DC: Armed Forces Institute of Pathology; 1995.

    Google Scholar 

  115. Donsbeck AV, et al. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999;34(4):295–304.

    Article  CAS  PubMed  Google Scholar 

  116. Simpson L, et al. Malignant primary cardiac tumors: review of a single institution experience. Cancer. 2008;112(11):2440–6.

    Article  PubMed  Google Scholar 

  117. Burke AP. Miscellaneous sarcomas. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 339.

    Google Scholar 

  118. Butany J, et al. Angiosarcoma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  119. Carpino F, et al. Angiosarcoma of the heart: structural and ultrastructural study. Eur Rev Med Pharmacol Sci. 2005;9(4):231–40.

    CAS  PubMed  Google Scholar 

  120. Butany J, Yu W. Cardiac angiosarcoma: two cases and a review of the literature. Can J Cardiol. 2000;16(2):197–205.

    CAS  PubMed  Google Scholar 

  121. Patel SD, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  122. Liu C, et al. Right atrial epithelioid angiosarcoma with multiple pulmonary metastasis confirmed by multimodality imaging-guided pulmonary biopsy: a case report and literature review. Medicine (Baltimore). 2018;97(30):e11588.

    Article  Google Scholar 

  123. El-Osta HE, et al. Unexplained hemopericardium as a presenting feature of primary cardiac angiosarcoma: a case report and a review of the diagnostic dilemma. J Thorac Oncol. 2008;3(7):800–2.

    Article  PubMed  Google Scholar 

  124. Cagle P, Dacic S. Mesenchymal tumours - epithelioid haemangioendothelioma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 123–4.

    Google Scholar 

  125. Abe D, et al. Life-threatening acute heart failure due to primary cardiac undifferentiated pleomorphic sarcoma. Intern Med. 2014;53(16):1775–7.

    Article  PubMed  Google Scholar 

  126. Tazelaar H, et al. Undifferentiated pleomorphic sarcoma. In: WHO classification of tumours of the lung, pleura, thymus and heart. 4th ed. Lyon: IARC Press; 2015.

    Google Scholar 

  127. Butany J, Fletcher CDM. Myxofibrosarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 334–5.

    Google Scholar 

  128. Burke AP, Tavora F. Osteosarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 333.

    Google Scholar 

  129. Aguilar CA, et al. A primary cardiac osteosarcoma: case report and review of the literature. J Cardiol Cases. 2013;7(2):e29–33.

    Article  PubMed  Google Scholar 

  130. Sun D, et al. Primary cardiac myxofibrosarcoma: case report, literature review and pooled analysis. BMC Cancer. 2018;18(1):512.

    Article  PubMed  PubMed Central  Google Scholar 

  131. Wang JG, et al. Primary cardiac myxofibrosarcoma: a case report and review of the literature. Tumori. 2012;98(6):165e–8e.

    Article  PubMed  Google Scholar 

  132. Burke AP. Leiomyosarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 336.

    Google Scholar 

  133. Orlandi A, et al. Cardiac sarcomas: an update. J Thorac Oncol. 2010;5(9):1483–9.

    Article  PubMed  Google Scholar 

  134. Varma T, Adegboyega P. Primary cardiac synovial sarcoma. Arch Pathol Lab Med. 2012;136(4):454–8.

    Article  PubMed  Google Scholar 

  135. Tazelaar H, Fletcher CDM. Synovial sarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 338.

    Google Scholar 

  136. Eswaran P, et al. Synovial sarcoma of the heart: a case report and literature review. J Cancer Res Ther. 2015;11(3):659.

    Article  PubMed  CAS  Google Scholar 

  137. Becker AE. Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol. 2000;21(4):317–23.

    Article  CAS  PubMed  Google Scholar 

  138. Butany J, Fletcher CDM. Rhabdomyosarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 337.

    Google Scholar 

  139. Grandmougin D, et al. Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival. Ann Thorac Surg. 2001;71(5):1438–41.

    Article  CAS  PubMed  Google Scholar 

  140. Kimura A, et al. A Mass filling the right atrium: primary cardiac rhabdomyosarcoma. Intern Med. 2018;57(24):3575–80.

    Article  PubMed  PubMed Central  Google Scholar 

  141. Burke AP, et al. Cardiac sarcomas - rhabdomyosarcoma. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004. p. 278–80.

    Google Scholar 

  142. Johansson L, Carlén B. Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations, and review of the literature. Virchows Arch. 1994;424(2):217–24.

    Article  CAS  PubMed  Google Scholar 

  143. Burke AP, Yi ES. Pulmonary artery intimal sarcoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 128–9.

    Google Scholar 

  144. Kerr KM. Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension. Nat Clin Pract Cardiovasc Med. 2005;2(2):108–12; quiz 113.

    Article  PubMed  Google Scholar 

  145. Maruo A, et al. Surgical experience for the pulmonary artery sarcoma. Ann Thorac Surg. 2006;82(6):2014–6.

    Article  PubMed  Google Scholar 

  146. Thakrar MV, Hirani N, Helmersen D. Recurrent pulmonary artery sarcoma treated with repeat pulmonary artery endarterectomy | A43. Interesting cases in interventional pulmonary: techniques and complications. Am J Respir Crit Care Med. 2010;181:A1587.

    Google Scholar 

  147. Corrin B, Nicholson AG. Pathology of the lungs. 3rd ed. London: Churchill Livingstone, Elsevier; 2011.

    Google Scholar 

  148. Kobayashi H, et al. A case of undifferentiated sarcoma in the superior vena cava and bilateral cervical veins. Am J Case Rep. 2018;19:1507–14.

    Article  PubMed  PubMed Central  Google Scholar 

  149. Gowda RM, Khan IA. Clinical perspectives of primary cardiac lymphoma. Angiology. 2003;54(5):599–604.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  151. Manlhiot C, et al. Post-transplant lymphoproliferative disorder in pediatric heart transplant recipients. J Heart Lung Transplant. 2010;29(6):648–57.

    Article  PubMed  Google Scholar 

  152. Maleszewski JJ, Jaffe ES. Cardiac lymphoma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 340–1.

    Google Scholar 

  153. Rolla G, Calligaris-Cappio F, Burke AP. Cardiac lymphomas. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004. p. 282–3.

    Google Scholar 

  154. Maleszewski JJ, Anavekar NS. Neoplastic pericardial disease. Cardiol Clin. 2017;35(4):589–600.

    Article  PubMed  Google Scholar 

  155. Thomason R, et al. 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 

  156. Andreani SM, et al. Extrapericardial solitary fibrous tumour of the pericardium. Eur J Cardiothorac Surg. 1998;14(1):98–100.

    Article  CAS  PubMed  Google Scholar 

  157. Restrepo CS, et al. Primary pericardial tumors. Radiographics. 2013;33(6):1613–30.

    Article  PubMed  Google Scholar 

  158. Tavora F, Travis WD. Tumours of the pericardium—solitary fibrous tumour. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015.

    Google Scholar 

  159. Tavora F, Fletcher CDM. Tumours of the pericardium—sarcomas. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 344–5.

    Google Scholar 

  160. Roggli V. Tumours of the pericardium—malignant mesothelioma. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 345–6.

    Google Scholar 

  161. Burke AP, Tavora F. Tumours of the pericardium - germ cell tumours. In: Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2015. p. 346–7.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Doris M. Rassl .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Rassl, D.M. (2019). Cardiac Tumours. In: Suvarna, S. (eds) Cardiac Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-24560-3_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-24560-3_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-24559-7

  • Online ISBN: 978-3-030-24560-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics