Primary Cardiac Malignancies: Epidemiology and Pathology

  • Stefania RizzoEmail author
  • Gaetano Thiene
  • Marialuisa Valente
  • Cristina Basso


Malignant cardiac tumors are exceedingly rare and mostly secondary. Primary malignant cardiac tumors are usually sarcomas (manly angiosarcoma, indifferentiated sarcoma and leiomysarcoma), followed by lymphomas. They can occur anywhere within the heart and the presenting symptoms are related to the localization, size, and histotype. Nowadays, diagnosis of cardiac tumors is made “ante mortem” thanks to echocardiography and other invasive or non-invasive techniques. Histopathology is mandatory to provide differential diagnosis among various cardiac masses and to characterize the precise tumor histotype to plan the best treatment. From the therapeutic viewpoint, surgical excision followed by chemotherapy and/or radiotherapy may prolong life. However, prognosis remains poor, therapies being all palliative, with the exception for lymphomas. The Chapter summarizes epidemiology, clinical features, histologic, and therapeutic aspects of malignant cardiac tumors.


  1. 1.
    Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG. WHO classification of tumours of the lung, thymus and heart. Pleura: IARC Press; 2015.Google Scholar
  2. 2.
    Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;77:107.CrossRefPubMedGoogle Scholar
  3. 3.
    Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol. 1997;80:671–82.CrossRefPubMedGoogle Scholar
  4. 4.
    Burke AP, Virmani R. Tumours of the heart and great vessels. 3rd ed. Washington, DC: Armed Forces Institute of Pathology; 1996.Google Scholar
  5. 5.
    Burke A, Jeudy Jr J, Virmani R. Cardiac tumours: an update. Heart. 2008;94:117–23.CrossRefPubMedGoogle Scholar
  6. 6.
    Basso C, Valente M, Thiene G. Cardiac tumor pathology. New York: Springer Humana Press; 2013.CrossRefGoogle Scholar
  7. 7.
    Basso C, Rizzo S, Valente M, Thiene G. Cardiac masses and tumours. Heart 2016; 102:1230-45.Google Scholar
  8. 8.
    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;117:1027–31.PubMedGoogle Scholar
  9. 9.
    McAllister HA, Hall RJ, Cooley DA. Tumors of the heart and pericardium. Curr Probl Cardiol. 1999;24:57–116.CrossRefPubMedGoogle Scholar
  10. 10.
    Basso C, Valente M, Thiene G. Tumori del cuore. Monografie di Cardiologia. Società Italiana di Cardiologia. Novate-Milan: Arti Grafiche Color Black; 2005.Google Scholar
  11. 11.
    Abraham KP, Reddy V, Gattuso P. Neoplasms metastatic to the heart: review of 3314 consecutive autopsies. Am J Cardiovasc Pathol. 1990;3:195–8.PubMedGoogle Scholar
  12. 12.
    Goldberg AD, Blankstein R, Padera RF. Tumors metastatic to the heart. Circulation. 2013;128:1790–4.CrossRefPubMedGoogle Scholar
  13. 13.
    Bussani R, De-Giorgio F, Abbate A, Silvestri F. Cardiac metastases. J Clin Pathol. 2007;60:27–34.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Bossert T, Gummert JF, Battellini R, Richter M, Barten M, Walther T, Falk V, Mohr FW. Surgical experience with 77 primary cardiac tumors. Interact Cardiovasc Thorac Surg. 2005;4:311–5.CrossRefPubMedGoogle Scholar
  15. 15.
    Centofanti P, Di Rosa E, Deorsola L, Dato GM, Patanè F, La Torre M, Barbato L, Verzini A, Fortunato G, di Summa M. Primary cardiac neoplasms: early and late results of surgical treatment in 91 patients. Ann Thorac Surg. 1999;68:1236–41.CrossRefPubMedGoogle Scholar
  16. 16.
    Burazor I, Aviel-Ronen S, Imazio M, Markel G, Grossman Y, Yosepovich A, Adler Y. Primary malignancies of the heart and pericardium. Clin Cardiol. 2014;37:582–8.PubMedGoogle Scholar
  17. 17.
    Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumours. Eur J Cardiothorac Surg. 1997;12:730–7.CrossRefPubMedGoogle Scholar
  18. 18.
    Neuville A, Collin F, Bruneval P, Parrens M, Thivolet F, Gomez-Brouchet A, Terrier P, de Montpreville VT, Le Gall F, Hostein I, Lagarde P, Chibon F, Coindre JM. Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas. Am J Surg Pathol. 2014;38:461–9.CrossRefPubMedGoogle Scholar
  19. 19.
    Fletcher CDM, Unni KK, Mertens F. World health organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002.Google Scholar
  20. 20.
    Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C. Soft-tissue sarcomas of adults: study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33:37–42.CrossRefPubMedGoogle Scholar
  21. 21.
    Reardon MJ, Walkes JC, Benjamin R. Therapy insight: malignant primary cardiac tumors. Nat Rev Cardiol. 2006;3:548–53.Google Scholar
  22. 22.
    Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. 1992;69:387–95.CrossRefPubMedGoogle Scholar
  23. 23.
    Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999;34:295–304.CrossRefPubMedGoogle Scholar
  24. 24.
    Rao U, Curtin J, Ryding A. Primary angiosarcoma of the heart. Heart. 2013;99:1878–9.CrossRefPubMedGoogle Scholar
  25. 25.
    Hermann MA, Shankerman RA, Edwards WD, Shub C, Schass HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg. 1992;103:655–64.Google Scholar
  26. 26.
    Poletti A, Cocco P, Valente M, Fasoli G, Chioin R, Thiene G. In vivo diagnosis of cardiac angiosarcoma by endomyocardial biopsy. Cardiovasc Pathol. 1993;2:89–91.CrossRefPubMedGoogle Scholar
  27. 27.
    Garcia JM, Gonzalez R, Jm S, Dominguez G, Vegazo IS, Gamallo C, Provencio M, España P, Bonilla F. Mutational status of K-ras and TP53 genes in primary sarcomas of the heart. Br J Cancer. 2000;82:1183–5.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Basso C, Stefani A, Calabrese F, Fasoli G, Valente M. Primary right atrial fibrosarcoma diagnosed by endocardial biopsy. Am Heart J. 1996;131:399–402.CrossRefPubMedGoogle Scholar
  29. 29.
    Mazzola A, Spano JP, Valente M, Gregorini R, Villani C, Di Eusanio M, Ciocca M, Minuti U, Giancola R, Basso C, Thiene G. Leiomyosarcoma of the left atrium mimicking a left atrial myxoma. J Thorac Cardiovasc Surg. 2006;131:224–6.CrossRefPubMedGoogle Scholar
  30. 30.
    Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol. 1997;60:91–4.CrossRefPubMedGoogle Scholar
  31. 31.
    Hui KS, Green LK, Schmidt WA. Primary cardiac rhabdomyosarcoma: definition of a rare entity. Am J Cardiovasc Pathol. 1988;2:19–29.PubMedGoogle Scholar
  32. 32.
    Hajar R, Roberts WC, Folger Jr GM. Embryonal botryoid rhabdomyosarcoma of the mitral valve. Am J Cardiol. 1986;57:376.CrossRefPubMedGoogle Scholar
  33. 33.
    Nicholson AG, Rigby M, Lincoln C, Meller S, Fisher C. Synovial sarcoma of the heart. Histopathology. 1997;30:349–52.CrossRefPubMedGoogle Scholar
  34. 34.
    Varma T, Adegboyega P. Primary cardiac synovial sarcoma. Arch Pathol Lab Med. 2012;136:454–8.CrossRefPubMedGoogle Scholar
  35. 35.
    Hazelbag HM, Szuhai K, Tanke HJ, Rosenberg C, Hogendoorn PC. Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype. Mod Pathol. 2004;17:1434–9.CrossRefPubMedGoogle Scholar
  36. 36.
    Petrich A, Cho SI, Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment and outcome patterns. Cancer 2011; 117: 581–9.Google Scholar
  37. 37.
    Bortolotti U, Calabro F, Loy M, Fasoli G, Altavilla G, Marchese D. Giant intrapericardial solitary fibrous tumor. Ann Thorac Surg. 1992;54:1219–20.CrossRefPubMedGoogle Scholar
  38. 38.
    Nilsson A, Rasmuson T. Primary pericardial mesothelioma: report of a patient and literature review. Case Rep Oncol. 2009;2:125–32.CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Sardar MR, Kuntz C, Patel T, Saeed W, Gnall E, Imaizumi S, Lande L. Primary pericardial mesothelioma unique case and literature review. Tex Heart Inst J. 2012;39:261–4.PubMedPubMedCentralGoogle Scholar
  40. 40.
    Luk A, Ahn E, Vaideeswar P, Butany JW. Pericardial tumors. Semin Diagn Pathol. 2008;25:47–53.CrossRefPubMedGoogle Scholar
  41. 41.
    Bruce CJ. Cardiac tumours: diagnosis and management. Heart. 2011;97:151–60.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Stefania Rizzo
    • 1
    Email author
  • Gaetano Thiene
    • 1
  • Marialuisa Valente
    • 1
  • Cristina Basso
    • 1
  1. 1.Department of Cardiac, Thoracic and Vascular Sciences, Pathological Anatomy-Cardiovascular PathologyAzienda Ospedaliera-University of Padova Medical SchoolPadovaItaly

Personalised recommendations