Cardiac tumors

  • Antonio F. Corno


Primary cardiac tumors are very rare clinical entities, with benign neoplasms occurring three to four times more often (70–90%) than malignant tumors (10–30%). Only series of myxomas in adults have been reported, while very few series involving different types of primary cardiac tumors in children have been reported. In this chapter only types of pediatric cardiac tumors occurring in the pediatric age will be considered. Metastatic cardiac neoplasms are not included. The ability to detect cardiac tumors with noninvasive diagnostic procedures even during the prenatal period has led to an apparent increase in this diagnosis, with incidences reported from 0.06% cases to 0.32%, while the reported autopsy series was limited to 0.027–0.08%.


Tuberous Sclerosis Cardiac Tumor Cardiac Myxoma Atrial Myxoma Cardiac Rhabdomyoma 
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  1. Agarwala B (2003) Rapid disappearance of a huge cardiac rhabdomyoma in an infant. Cardiol Young 13:173–174PubMedCrossRefGoogle Scholar
  2. Alkalay AL, Ferry DA, Lin B, Fink BW, Pomerance JJ (1987) Spontaneous regression of cardiac rhabdomyoma in tuberous sclerosis. Clin Pediatr 26:532–535CrossRefGoogle Scholar
  3. Arciniegas E, Hakimi M, Farooki ZQ, Truccone NJ, Green EW (1980) Primary cardiac tumors in children. J Thorac Cardiovasc Surg 79:582–591PubMedGoogle Scholar
  4. Becker AE (2000) Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 21:317–323PubMedCrossRefGoogle Scholar
  5. Bini RM, Westaby S, Bargeron LM, Pacifico AD, Kirklin JW (1983) Investigation and management of primary cardiac tumors in infants and children. J Am Coll Cardiol 2:351–357PubMedCrossRefGoogle Scholar
  6. Bittner HB, Sharma AD, Landolfo KP (2000) Surgical resection of an intracardiac rhabdomyoma. Ann Thorac Surg 70:2156–2158PubMedCrossRefGoogle Scholar
  7. Bloomberg RD, Butany JW, Cusimano RJ, Leask RL (2003) Primary cardiac sarcoma involving the pulmonary artery and valve. Can J Cardiol 19:843–847PubMedGoogle Scholar
  8. Bosi G, Lintermans JP, Pellegrino PA, Svaluto-Moreolo G, Vliers A (1996) The natural history of cardiac rhabdomyoma with and without tuberous sclerosis. Acta Paediatr 85:928–931PubMedCrossRefGoogle Scholar
  9. Cho JM, Danielson GK, Puga FJ, Dearani JA, McGregor CGA, Tazelaar HD, Hagler DJ (2003) Surgical resection of ventricular cardiac fibromas: early and late results. Ann Thorac Surg 76:1929–1934PubMedCrossRefGoogle Scholar
  10. Corno AF, de Simone G, Catena G, Marcelletti C (1984) Cardiac rhabdomyoma: surgical treatment in the neonate. J Thorac Cardiovasc Surg 87:725–731PubMedGoogle Scholar
  11. Corno AF, Marianeschi S, Giannico S, Parisi F, Marino B (1989) Cardiac rhabdomyoma in infancy. CI Res 37:92AGoogle Scholar
  12. Corno AF (1996) Fetal echocardiography for neonatal cardiac tumors. J Am Coll Cardiol 28:1080PubMedGoogle Scholar
  13. Costas C, Williams RL, Fortune RL (1986) Intracardiac teratoma in an infant. 7:179–181Google Scholar
  14. D’Addario V, Pinto V, Di Naro E, Del Bianco A, Di Cagno L, Volpe P (2002) Prenatal diagnosis and postnatal outcome of cardiac rhabdomyomas. J Perinat Med 30:170–175PubMedCrossRefGoogle Scholar
  15. Di Liang C, Ko SF, Huang SC (2000) Echocardiographic evaluation of cardiac rhabdomyoma in infants and children. J Clin Ultrasound 28:381–386PubMedCrossRefGoogle Scholar
  16. Duncan WJ, Freedom RM, Rowe RD (1982) Space-occupying lesions of the myocardium: role of two-dimensional echocardiography in detection of cardiac tumors in children. Am Heart J 104:780–785PubMedCrossRefGoogle Scholar
  17. Elderkin RA, Radford DJ (2002) Primary cardiac tumours in a paediatric population. J Paediatr Child Health 38:173–177PubMedCrossRefGoogle Scholar
  18. Engle MA, Ebert PA, Redo SF (1974) Recurrent ventricular tachycardia due to respectable cardiac tumor. Circulation 50:1052–1057PubMedCrossRefGoogle Scholar
  19. Eslami-Varzaneh F, Brun EA, Sears-Rogan P (2003) An unusual case of multiple papillary fibroelastoma, review of literature. Cardiovasc Pathol 12: 170–173PubMedCrossRefGoogle Scholar
  20. Freedom RM, Lee KJ, MacDonald C, Taylor G (2000) Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol 21:299–316PubMedCrossRefGoogle Scholar
  21. Fyler DC, Buckley LP, Hellenbrand WE, Cohn HE (1980) Report of the New England Regional Infant Care Program. Pediatrics 65(Suppl):375–461Google Scholar
  22. Gamzu R, Achiron R, Hegesh J, Weiner E, Tepper R, Nir A, Rabinowitz R, Auslander R, Yagel S, Zalel Y, Zimmer E (2002) Evaluating the risk of tuberous sclerosis in cases with prenatal diagnosis of cardiac rhabdomyoma. Prenat Diagn 22:1044–1047PubMedCrossRefGoogle Scholar
  23. Geha AS, Weidman WH, Soule EH, McGoon DC (1967) Intramural ventricular cardiac fibroma. Circulation 36:427–440PubMedCrossRefGoogle Scholar
  24. Geipel A, Krapp M, Germer U, Becker R, Gembruch U (2001) Ultrasound Obstet Gynecol 17:17–21PubMedCrossRefGoogle Scholar
  25. Golding R, Reed G (1967) Rhabdomyoma of the heart. N Engl J Med 276:957–960PubMedCrossRefGoogle Scholar
  26. Gowdamarajan A, Michler RE (2002) Therapy for primary cardiac tumors: ppis there a role for heart transplantation? Curr Opin Cardiol 15:121–125CrossRefGoogle Scholar
  27. Harding CO, Pagon RA (1990) Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am J Med Genet 37:443–446PubMedCrossRefGoogle Scholar
  28. Harinck E, Moulaert AJMG, Rohmer J, Brom GA (1974) Cardiac rhabdomyoma in infancy. Acta Paediatr Scand 63:283–286PubMedCrossRefGoogle Scholar
  29. Hoffmeier A, Scheid HH, Tjan TD, Schneider M, Kerber S, Schmidt C, Schmid C (2003) Ex situ resection of primary cardiac tumors. Thorac Cardiovasc Surg 51:99–101PubMedCrossRefGoogle Scholar
  30. Houser S, Forbes N, Stewart S (1980) Rhabdomyoma of the heart: a diagnostic and therapeutic challenge. Ann Thorac Surg 29:373–377PubMedCrossRefGoogle Scholar
  31. Jiang ZY, Pircova A, Sekarski N, Hack I, Laurini R, Janzer R, Payot M (2000) Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis. Pediatr Cardiol 21:165–169PubMedCrossRefGoogle Scholar
  32. Kiaffas MG, Powell AJ, Geva T (2002) magnetic resonance imaging evaluation of cardiac tumor characteristics in infants and children. Am J Cardiol 89:1229–1233PubMedCrossRefGoogle Scholar
  33. Kim JT, Baek WK, Kim KH, Yoon YH, Kim DH, Lim HK (2003) A primary cardiac sarcoma preoperatively presented as a benign left atrial myxoma. Yonsei Med J 44:530–533PubMedGoogle Scholar
  34. Lam KY, Dickens P, Chan AC (1993) Tumors of the heart: a 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 117:1027–1031PubMedGoogle Scholar
  35. Lethor JP, de Moor M (2001) Multiple cardiac tumors in the fetus. Circulation 103:E55PubMedCrossRefGoogle Scholar
  36. Ludomirsky A (1998) Cardiac tumors. In: Bricker JT, Fisher DJ (Eds) The science and practice of pediatric cardiology, 9th ed. Williams & Wilkins; pp 1885–1893Google Scholar
  37. Mair DD, Titus JL, Davis GD, Ritter DG (1977) Cardiac rhabdomyoma simulating mitral atresia. Chest 71:102–105PubMedCrossRefGoogle Scholar
  38. Marin-Garcia J, Fitch CW, Shenefelt RE (1984) Primary right ventricular tumor (fibroma) simulating cyanotic heart disease in a newborn. J Am Coll Cardiol 3:868–871PubMedCrossRefGoogle Scholar
  39. Marino B, Corno AF, Carotti A, Pasquini L, Giannico S, Guccione P, Bevilacqua M, De Simone G, Marcelletti C (1990) Pediatric cardiac surgery guided by echocardiography. Scand J Thorac Cardiovasc Surg 24:197–201PubMedCrossRefGoogle Scholar
  40. Marx GR, Moran AM (2000) Cardiac tumors. In: Moss and Adams “ Heart disease in infants, children and adolescents”, 6th ed. Lippincott, Williams and Wilkins, pp 1432–1445Google Scholar
  41. McMahon CJ, Ayres NA, Lewin MB (2001) Cardiac rhabdomyoma: a report of alternative strategies to surgical resection. Cardiol Young 11:670–672PubMedCrossRefGoogle Scholar
  42. Padalino MA, Basso C, Svaluto-Moreolo G, Thiene G, Stellin G (2003) Left atrial Myxoma in a child: case report and review of the literature. Cardiovasc Pathol 12:233–236PubMedCrossRefGoogle Scholar
  43. Percell RL, Henning RJ, Siddique Patel M (2003) Atrial myxoma: case report and a review of the literature. Heart Dis 5:224–230PubMedCrossRefGoogle Scholar
  44. Pipitone S, Mongiovi M, Grillo R, Gagliano S, Sperandeo V (2002) Cardiac rhabdomyoma in intrauterine life: clinical features and natural history: a case series and review of published reports. Ital Heart J 3:48–52PubMedGoogle Scholar
  45. Reece IJ, Houston AB, Pollock JCS (1983) Interventricular fibroma: echocardiographic diagnosis and successful surgical removal in infancy. Br Heart J 50:590–591PubMedCrossRefGoogle Scholar
  46. Riggs TW, Sholl JS, Ilbawi M, Gardner T (1984) In utero diagnosis of pericardial tumour with successful surgical repair. Pediatr Cardiol 5:23–26PubMedCrossRefGoogle Scholar
  47. Schmaltz AA, Apitz J (1982) Primary rhabdomyosarcoma of the heart. Pediatr Cardiol 2:73–75PubMedCrossRefGoogle Scholar
  48. Schaher RM, Farina M, Alley R, Hansen P, Bishop M (1972) Congenital subaortic stenosis in infancy caused by rhabdomyoma of the left ventricle. J Thorac Cardiovasc Surg 63:157–163Google Scholar
  49. Shiono J, Horigome H, Yasui S, Miyamoto T, Takahashi-Igari M, Iwasaki N, Matsui A (2003) Electrocardiographic changes in patients with cardiac rhabdomyomas associated with tuberous sclerosis. Cardiol Young 13:258–263PubMedGoogle Scholar
  50. Stellingwerff GC, Hess J, Bogers AJ (1999) Left ventricular rhabdomyoma. J Cardiovasc Surg 40:131–133Google Scholar
  51. Stiller B, Hetzer R, Mayer R, Dittrich S, Pees C, Alexi-Meskishvili V, Lange PE (2001) Primary cardiac tumours: when is surgery necessary? Eur J Cardiothorac Surg 20:1002–1006PubMedCrossRefGoogle Scholar
  52. Takach TJ, Reul GJ, Ott DA, Cooley DA (1996) Primary cardiac tumors in infants and children: immediate and long term operative results. Ann Thorac Surg 62:559–564PubMedCrossRefGoogle Scholar
  53. Tworetzky W, McElhinney DB, Margossian R, MoonGrady AJ, Sallee D, Goldmuntz E, van der Velde ME, Silverman NH, Allan LD (2003) Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol 92:487–489PubMedCrossRefGoogle Scholar
  54. Val-Bernal JF, Acebo E, Gomez-Roman JJ, Garijo MF (2003) Anticipated diagnosis of left atrial myxoma following histological investigation of limb embolectomy specimens: a report of two cases. Pathol Int 53:489–494PubMedCrossRefGoogle Scholar
  55. Vander Salm TJ (2000) Unusual primary tumours of the heart. Semin Thorac Cardiovasc Surg 12:89–100Google Scholar
  56. Vaughan CJ, Veugelers M, Basson CT (2001) Tumors and the heart: molecular genetic advances. Curr Opin Cardiol 16:195–200PubMedCrossRefGoogle Scholar
  57. Webb DW, Thomas RD, Osborne JP (1993) Cardiac rhabdomyomas and their association with tuberous sclerosis. Arch Dis Child 68:367–370PubMedCrossRefGoogle Scholar
  58. Webb DW, Osborne JP (1995) Tuberous sclerosis. Arch Dis Child 72:471–474PubMedCrossRefGoogle Scholar
  59. Williams WG, Trusler GA, Fowler RS, Scott ME, Mustard WT (1972) Left ventricular myocardial fibroma: a case report and review of cardiac tumors in children. J Pediatr Surg 7:324–328PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2004

Authors and Affiliations

  • Antonio F. Corno
    • 1
  1. 1.Department of Cardiovascular SurgeryCentre Hospitalier Universitaire Vaudois (CHUV)LausanneSwitzerland

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