Papillary Fibroelastoma of the Heart

Authors

  • Vassil Gegouskov
  • Alexander Kadner
  • Lars Engelberger
  • Thierry Carrel
  • Hendrik Tevaearai

DOI:

https://doi.org/10.1532/HSF98.20081091

Abstract

Background: Fibroelastoma is a rare cardiac tumor that was originally described typically from autopsy findings. Thanks to improved imaging modalities, such tumors are today relatively easy to detect and therefore are actively searched for in patients with unclear embolic events. We present the cases of 2 patients recently treated in our clinic and review the recent literature pertaining to fibroelastomas.

Methods and Results: An electronic PubMed search revealed 186 cases reported between 1994 and 2003. Ninety-seven percent of the reported fibroelastomas were diagnosed in living patients in their fourth and fifth decades of life. The majority (86%) were symptomatic, with stroke, transient ischemic attack, myocardial infarction, and angina pectoris being the most commonly described. Echocardiography was the typical diagnosis modality. Surgical resection was completed in 95% of the cases.

Conclusions: Although cardiac papillary fibroelastomas are rare and benign tumors, they cannot be considered as harmless endothelial lesions, because related embolic events are frequent and primarily involve adults in their active period of life. Echocardiography must therefore consider fibroelastoma in the differential diagnosis of every unclear systemic embolic event, especially because surgical resection can be considered as curative.

References

Alawi A, Kassabian EB, Ashoush R, Jebara VA. 2002. Aortic valve papillary fibroelastoma. Cardiovasc Surg 10:65-7.nAnderson KR, Fiddler GI, Lie JT. 1977. Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E. Mayo Clin Proc 52:665-9.nBoone SA, Campagna M, Walley VM. 1992. Lambl's excrescences and papillary fibroelastomas: Are they different? Can J Cardiol 8:372-6.nCheitlin MD, McAllister HA, de Castro CM. 1975. Myocardial infarction without atherosclerosis. JAMA 231:951-9.nChitwood WR Jr. 1988. Cardiac neoplasms: current diagnosis, pathology, and therapy [review]. J Card Surg 3:119-54.nColucci WS, Schoen FJ, Braunwald E. 2001. Primary tumors of the heart. In: Braunwald E, Zipes DP, Libby P, eds. Heart disease. 6th ed. Philadelphia, Pa: WB Saunders. p 1807-22.nDennis MA, Appareti K, Manco-Johnson ML, et al. 1985. The echocardiographic diagnosis of multiple fetal cardiac tumors. Ultrasound Med 4:327.nEckstein FS, Schafers HJ, Grote J, Mugge A, Borst HG. 1995. Papillary fibroelastoma of the aortic valve presenting with myocardial infarction. Ann Thorac Surg 60:206-8.nFishbein MC, Ferrans VJ, Roberts WC. 1975. Endocardial papillary elastofibromas. Histologic, histochemical, and electron microscopical findings. Arch Pathol 99:335-41.nGeorghiou GP, Erez E, Vidne BA, Aravot D. 2003. Tricuspid valve papillary fibroelastoma: an unusual cause of intermittent dyspnea. Eur J Cardiothorac Surg 23:429-31.nGowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. 2003. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 146:404-10.nGully C, Benghanem MM, Motebassem R, Sagan C. 1998. Clinical expression of papillary fibroelastoma. Apropos of a case [in French]. Arch Mal Coeur Vaiss 91:777-82.nHeath D, Best PV, Davis BT. 1961. Papilliferous tumours of the heart valves. Br Heart J 23:20-4.nHoward RA, Aldea GS, Shapira OM, Kasznica JM, Davidoff R. 1999. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 68:1881-5.nHynes MS, Veinot JP, Chan KL. 2002. Occurrence of a second primary papillary fibroelastoma. Can J Cardiol 18:753-6.nKouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. 2003. Cardiac tumor. In: Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. 3rd ed. Philadelphia, Pa: Elsevier Science. p 1679-99.nKurup AN, Tazelaar HD, Edwards WD, et al. 2002. Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990 to 2000). Hum Pathol 33:1165-9.nLichtenstein HL, Lee JC, Stewart S. 1979. Papillary tumor of the heart: incidental finding at surgery. Hum Pathol 10:473-5.nMcAllister HA, Fenoglio JJ. 1978. Tumors of the cardiovasculary system. In: Atlas of tumor pathology. 2nd series, Washington DC: Armed Forces Institute of Pathology. Fascicle 15:20-5.nMeng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. 2002. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol 84:69-75.nMinatoya K, Okabayashi H, Yokota T, Hoover EL. 1996. Cardiac papillary fibroelastomas: rationale for excision. Ann Thorac Surg 62:1519-21.nNeerukonda SK, Jantz RD, Vijay NK, Narrod JA, Schoonmaker FW. 1991. Pulmonary embolization of papillary fibroelastoma arising from the tricuspid valve. Tex Heart Inst J 18:132-5.nOkada K, Sueda T, Orihashi K, Watari M, Matsuura Y. 2001. Cardiac papillary fibroelastoma on the pulmonary valve: a rare cardiac tumor. Ann Thorac Surg 71:1677-9.nOostenbrug LE, Ottervanger JP, Dompeling EC, Sie TH, Beukema WP. 2001. A bleeding disorder caused by a cardiac tumor: case report. Neth J Med 58:232-5.nRbaibi A, Bonnevie L, Guiraudet O, et al. 2002. Importance of transesophageal echocardiography and computed tomography in the differential diagnosis of a case of papillary fibroelastoma revealed by a neurologic accident [in French]. Arch Mal Coeur Vaiss 95:601-5.nRubin MA, Snell JA, Tazelaar HD, Lack EE, Austenfeld JL, Azumi N. 1995. Cardiac papillary fibroelastoma: an immunohistochemical investigation and unusual clinical manifestations. Mod Pathol 8:402-7.nSaad RS, Galvis CO, Bshara W, Liddicoat J, Dabbs DJ. 2001. Pulmonary valve papillary fibroelastoma. A case report and review of the literature. Arch Pathol Lab Med 125:933-4.nSalyer WR, Page DL, Hutchins GM. 1975. The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J 89:4-17.nSavino JS, Weiss SJ. 1995. Images in clinical medicine. Right atrial tumor. N Engl J Med 333:1608.nShahian DM, Labib SB, Chang G. 1995. Cardiac papillary fibroelastoma. Ann Thorac Surg 59:538-41.nSun JP, Asher CR, Yang XS, et al. 2001. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 103:2687-93.nYater WM. 1931. Tumors of the heart and pericardium: pathology, symptomatology and report of nine cases. Arch Intern Med 48:627-66.nYee HC, Nwosu JE, Lii AD, Velasco M, Millman A. 1997. Echocardographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 80:811-4.n

Published

2008-12-15

How to Cite

Gegouskov, V., Kadner, A., Engelberger, L., Carrel, T., & Tevaearai, H. (2008). Papillary Fibroelastoma of the Heart. The Heart Surgery Forum, 11(6), E333-E339. https://doi.org/10.1532/HSF98.20081091

Issue

Section

Article