Skip to main content

Cardiac Tumors: From Autoptic Observations to Surgical Pathology in the Era of Advanced Cardiac Imaging

  • Chapter
  • First Online:
Cardiac Tumor Pathology

Abstract

Although the term tumor generally recalls the idea of “cancer,” at cardiac level most of primary tumors are biologically benign and malignancy is mostly hemodynamic, due to obstruction of the blood flow because of intracavitary growth and embolism following neoplastic fragmentation with ischemic damage of several organs. The first book on cardiac tumors was published in 1945 by Ivan Mahaim, Professor at the University of Lausanne and was a collection of postmortem observations and a thorough review of the literature. While treating atrial myxoma (“Le polype du coeur”), the most frequent cardiac tumor (nearly two-thirds of primary heart neoplasms), he said “…surgical resection of atrial polyp encounters apparently insurmountable difficulties. However, we should not give up because of this feeling. In any field of science, with technological progress, the impossible is just a moment during the evolution of our powers. As Mummery said about alpinism, the inaccessible peak becomes an easy route for ladies…”. In fact, some years later the era of “surgical pathology” started with the advent of cardiac imaging and open heart surgery in the 1960s, when cardiac neoplasms were diagnosed during life and not only in the autopsy room and became surgically resectable with excellent long-term survival. The historical watershed in the diagnosis and treatment came in the 1980s, with the advent of non-invasive imaging, i.e., echocardiography, which, together with computed tomography and cardiac magnetic resonance, substantially improved non-invasive diagnosis and subsequent treatment. It is possible to easily visualize cardiac tumors at the first onset of symptoms or even incidentally, during routine diagnostic procedures, and send the patient promptly to the surgeon for resection with a nearly 100 % success in the benign forms. Nowadays, the pathologist is on call to achieve the in vivo diagnosis on endomyocardial or surgical biopsies, by establishing the nature (benign, malignant, or non-neoplastic, usually thrombi or vegetations) and the histotype, and to make the differential diagnosis with secondary tumors. In this chapter we will come across single case observations that witness the advances in the clinical diagnosis and therapy of cardiac tumors.

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. Columbus MR. De Re Anatomica, Libri XV. Venice: N Bevilacque,1559, p 269.

    Google Scholar 

  2. Prichard RW. Tumors of the heart: review of the subject and report of 150 cases. Arch Pathol. 1951;51:98–128.

    CAS  Google Scholar 

  3. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol. 1980;101:219–40.

    CAS  PubMed  Google Scholar 

  4. Mahaim I. Le tumeurs et les polypes du coeur: etude anatomoclinique. Paris: Masson; 1945.

    Google Scholar 

  5. Goldberg HP, Glenn F, Dotter CT, Steinberg I. Myxoma of the left atrium: diagnosis made during life with operative and post-mortem findings. Circulation. 1952;6:762–7.

    Article  CAS  PubMed  Google Scholar 

  6. Crafoord C. Panel discussion on late results of mitral commissurotomy. In: Lam CR, editor. International symposium on cardiovascular surgery. Philadelphia, PA: Saunders; 1955. p. 161–78

    Google Scholar 

  7. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg. 1997;12:730–7.

    Article  CAS  PubMed  Google Scholar 

  8. Basso C, Valente M, Casarotto D, Thiene G. Cardiac lithomyxoma. Am J Cardiol. 1997;80:1249–51.

    Article  CAS  PubMed  Google Scholar 

  9. 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.

    Article  Google Scholar 

  10. Valente M, Cocco P, Thiene G, Casula R, Poletti A, Milanesi O, Fasoli G, Livi U. Cardiac fibroma and heart transplantation. J Thorac Cardiovasc Surg. 1993;106:1208–12.

    CAS  PubMed  Google Scholar 

  11. Hoffmann U, Globits S, Schima W, Loewe C, Puig S, Oberhuber G, Frank H. Usefulness of magnetic resonance imaging of cardiac and paracardiac masses. Am J Cardiol. 2003;92:890–5.

    Article  PubMed  Google Scholar 

  12. Basso C, Fasoli G, Casarotto D, Thiene G. Unusual left ventricular mass. Circulation. 1998;98:1036–7.

    Article  CAS  PubMed  Google Scholar 

  13. Basso C, Bottio T, Rubino M, Ruffatti A, Pittarello D, Thiene G, Gerosa G. Antiphospholipid sindrome and right atrial mass. J Thorac Cardiovasc Surg. 2005;130:1462–3.

    Article  PubMed  Google Scholar 

  14. Vida V, Biffanti R, Thiene G, Stellin G, Milanesi O, Basso C. Left ventricular mass after treatment with chemotherapic drugs. Circulation. 2004;109:e300–1.

    Article  PubMed  Google Scholar 

  15. Casarotto D. Mixoma dell’atrio sinistro. Chirurgia Triveneta. 1971;11:279–87.

    Google Scholar 

  16. Thiene G, Miraglia G, Menghetti L, Nava A, Rossi L. Multiple lesions of the conduction system in a case of cardiac rhabdomyosarcoma with complex arrhythmias. An anatomic and clinical study. Chest. 1976;70:378–81.

    Article  CAS  PubMed  Google Scholar 

  17. Casarotto D, Bortolotti U, Russo D, Betti D, Schivazappa L, Thiene G. Surgical removal of a left atrial myxoma during pregnancy. Chest. 1979;75:390–2.

    Article  CAS  PubMed  Google Scholar 

  18. Ballotta E, Thiene G, Valente M, Deriu GP. Mixoma dell’atrio sinistro rivelato da un’occlusione acuta del carrefour aortico. G Ital Cardiol. 1979;9:627–30.

    CAS  PubMed  Google Scholar 

  19. Stritoni P, Daliento L, Fasoli G, Schivazappa L, Chioin R, Fusaro A, Casarotto D, Bortolotti U, Valente M, Thiene G. Mixoma atriale. Esperienza clinica, diagnostico-strumentale, chirurgica e patologica in 13 casi. G Ital Cardiol. 1979;9:1001–16.

    CAS  PubMed  Google Scholar 

  20. Gallucci V, Stritoni P, Fasoli G, Thiene G. Giant blood cyst of tricuspid valve. Successful excision in an infant. Br Heart J. 1976;38:990–2.

    Article  CAS  PubMed  Google Scholar 

  21. Bortolotti U, Mazzucco A, Valfrè C, Valente M, Pennelli N, Gallucci V. Right ventricular myxoma: review of the literature and report of two patients. Am Thorac Surg. 1982;33:277–84.

    Article  CAS  Google Scholar 

  22. Stellin G, Bortolotti U, Valfré C, Mazzucco A, Thiene G, Cavarzerani A, Gallucci V. Mural papilloma of the left ventricle and floppy mitral valve: report of an unusual association. Texas Heart Inst J. 1983;10:89–92.

    CAS  Google Scholar 

  23. Valente M. Structural profile of cardiac myxoma. Appl Pathol. 1983;1:251–63.

    CAS  PubMed  Google Scholar 

  24. Valente M, Montaguti A. Il mixoma cardiaco nell’adolescenza. Studio anatomo-patologico in tre pazienti. Pathologica. 1984;76:323–32.

    CAS  PubMed  Google Scholar 

  25. Livi U, Bortolotti U, Milano A, Valente M, Prandi A, Frugoni C, De Mozzi P, Valfré C, Mazzucco A, Gallucci V. Cardiac myxomas: results of 14 years experience. Thorac Cardiovasc Surg. 1984;32:143–7.

    Article  CAS  PubMed  Google Scholar 

  26. Thiene G, Valente M. I tumori primitivi del cuore e del pericardio nell’esperienza dell’Istituto di Anatomia Patologica dell’Università di Padova (1972–1984). In: Pansini R, editors. Progressi Clinici: Medicina. Padova: Piccin Ed., 1985;2/2–3: p. 169–207.

    Google Scholar 

  27. Bortolotti U, Mazzucco A, Rubino M, Maraglino G, Sturaro M, Milano A, Stellin G, Thiene G, Gallucci V. I mixomi dell’atrio destro. Tecnica operatoria, risultati e follow-up a distanza. Chir Torac. 1989;42:371–2.

    Google Scholar 

  28. Mazzucco A, Bortolotti U, Thiene G, Dan M, Stritoni P, Scutari M, Stellin G. Left ventricular papillary fibroelastoma with coronary embolization. Eur J Cardio-Thorac Surg. 1989;3:471–3.

    Article  CAS  Google Scholar 

  29. De Dominicis E, Frigiola A, Thiene G, Menicanti L, Bozzola L, Finocchi G. Subaortic stenosis by solitary rhabdomyoma. Successful excision in an infant following 2D echocardiogram and Doppler diagnosis. Chest. 1989;95:470–2.

    Article  PubMed  Google Scholar 

  30. Bortolotti U, Maraglino G, Rubino M, Santini F, Mazzucco A, Milano A, Fasoli G, Livi U, Thiene G, Gallucci V. Surgical excision of intracardiac myxomas: a 20-year follow-up. Ann Thorac Surg. 1990;49:449–53.

    Article  CAS  PubMed  Google Scholar 

  31. Bortolotti U, Faggian G, Mazzucco A, Milano A, Thiene G, Fasoli G, Gallucci V. Right atrial myxoma originating from the inferior vena cava. Ann Thorac Surg. 1990;49:1000–2.

    Article  CAS  PubMed  Google Scholar 

  32. Cocco P, Valente M, Thiene G. I tumori primitivi del cuore nell’era cardiochirurgica: dall’autopsia al trapianto cardiaco. Riv Anat Pat Oncol. 1991;49–50:135–72.

    Google Scholar 

  33. Mazzucco A, Faggian G, Bortolotti U, Bonato R, Pittarello D, Centonze G, Thiene G. Embolizing papillary fibroelastoma of the mitral valve. Texas Heart Inst J. 1991;18:62–6.

    CAS  Google Scholar 

  34. Valente M, Basso C, Thiene G, Bressan M, Stritoni P, Cocco P, Fasoli G. Fibroelastic papilloma: a not-so-benign cardiac tumor. Cardiovasc Pathol. 1992;1:161–6.

    Article  Google Scholar 

  35. 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.

    Article  CAS  PubMed  Google Scholar 

  36. Basso C, Poletti A, Valente M, Thiene G. Diagnosi di tumore cardiaco tramite biopsia endomiocardica. In: Baroldi G, Thiene G, editors. Testo Atlante Biopsia Endomiocardica. Padova: Piccin Ed; 1996. p. 178–96.

    Google Scholar 

  37. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardio-thorac Surg. 1997;12:730–8.

    Article  CAS  Google Scholar 

  38. Scalia D, Basso C, Rizzoli G, Lupia M, Budano S, Thiene G, Venturini A. Should right-sided fibroelastomas be operated upon? J Heart Valve Dis. 1997;6:647–50.

    CAS  PubMed  Google Scholar 

  39. Basso C, Barbazza R, Thiene G. Lipomatous hypertrophy of the atrial septum. Circulation. 1998;97:1423.

    Article  CAS  PubMed  Google Scholar 

  40. Testolin L, Basso C, Pittarello D, Casarotto D, Valente M. Cardiogenic shock due to metastatic cardiac lymphoma: still a diagnostic and therapeutic challenge. Eur J Cardiothorac Surg. 2001;19:365–8.

    Article  CAS  PubMed  Google Scholar 

  41. Bottio T, Basso C, Rizzoli G, Casarotto D, Thiene G. Case report: fibroelastoma of the papillary muscle of the mitral valve: diagnostic implications and review of the literature. J Heart Valve Dis. 2002;11:288–91.

    PubMed  Google Scholar 

  42. Basso C, Valente M, Thiene G. Aspetti anatomo-clinici dei tumori del cuore e del pericardio. In: Crepaldi G, Baritussio A, editors. Trattato di Medicina Interna: Malattie del cuore e dei vasi. Padova: Piccin Editore; 2002. p. 757–68.

    Google Scholar 

  43. Padalino MA, Basso C, Thiene G, Stellin G. Images in cardiovascular medicine: Giant right ventricular fibroma in an infant. Circulation. 2002;106:386.

    Article  PubMed  Google Scholar 

  44. Padalino MA, Basso C, Moreolo GS, Thiene G, Stellin G. Left atrial myxoma in a child: case report and review of the literature. Cardiovasc Pathol. 2003;12:233–6.

    Article  PubMed  Google Scholar 

  45. Basso C, Bottio T, Valente M, Bonato R, Casarotto D, Thiene G. Primary cardiac valve tumours. Heart. 2003;89:1259–60.

    Article  CAS  PubMed  Google Scholar 

  46. Rizzoli G, Bottio T, Pittarello D, Napodano M, Thiene G, Basso C. Atrial septal mass: transesophageal echocardiographic assessment. J Thorac Cardiovasc Surg. 2004;128:767–9.

    Article  PubMed  Google Scholar 

  47. Bottio T, Pittarello D, Bonato R, Thiene G, Gerosa G, Casarotto D, Basso C. Echocardiographic diagnosis of aortic valve papillary fibroelastoma. Tex Heart Inst J. 2004;31:322–323

    Google Scholar 

  48. Vida V, Cerutti A, Thiene G, Stellin S, Milanesi O, Basso C. Calcified mass in the right atrium. Ann Thorac Surg. 2005;79:717.

    Article  PubMed  Google Scholar 

  49. Padalino M, Basso C, Milanesi O, Vida VL, Svaluto Moreolo G, Thiene G, Stellin G. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg. 2005;129:1358–63.

    Article  PubMed  Google Scholar 

  50. 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.

    Article  PubMed  Google Scholar 

  51. Thiene G, Valente M, Lombardi M, Basso C. Tumours of the heart. In: Camm JA, Luscher TF, Serruys PV, editors. ESC textbook of cardiovascular medicine. Oxford: Oxford University Press; 2009.

    Google Scholar 

  52. Rickelt S, Rizzo S, Doerflinger Y, Zentgraf H, Basso C, Gerosa G, Thiene G, Moll R, Franke WW. A novel kind of tumor type-characteristic junction: plakophilin-2 as a major protein of adherens junctions in cardiac myxomata. Mod Pathol. 2010;23:1429–37.

    Article  CAS  PubMed  Google Scholar 

  53. Padalino MA, Vida VL, Bhattarai A, Reffo E, Milanesi O, Thiene G, Stellin G, Basso C. Giant intramural left ventricular rhabdomyoma in a newborn. Circulation. 2011;124:2275–7.

    Article  PubMed  Google Scholar 

  54. Perazzolo M, Thiene G, De Lazzari M, Calabrese F, Lacognata C, Rizzo S, Cacciavillani L, Tona F, Corbetti F, Iliceto S, Basso C. Concealed metastatic lung carcinoma presenting as acute coronary syndrome with progressive conduction abnormalities. Circulation. 2012;125:e499–502.

    Article  PubMed  Google Scholar 

  55. Padalino MA, Vida VL, Boccuzzo G, Tonello M, Sarris GE, Berggren H, Comas JV, Di Carlo D, Di Donato RM, Ebels T, Hraska V, Jacobs JP, Gaynor JW, Metras D, Pretre R, Pozzi M, Rubay J, Sairanen H, Schreiber C, Maruszewski B, Basso C, Stellin G. Surgery for primary cardiac tumors in children: early and late results in a multicenter European congenital heart surgeons association study. Circulation. 2012;126:22–30.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Gaetano Thiene M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Thiene, G., Valente, M., Basso, C. (2013). Cardiac Tumors: From Autoptic Observations to Surgical Pathology in the Era of Advanced Cardiac Imaging. In: Basso, C., Valente, M., Thiene, G. (eds) Cardiac Tumor Pathology. Current Clinical Pathology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-143-1_1

Download citation

  • DOI: https://doi.org/10.1007/978-1-62703-143-1_1

  • Published:

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-62703-142-4

  • Online ISBN: 978-1-62703-143-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics