Nonvalvular right atrial papillary fibroelastoma.

We present the case of a 61-year-old woman with a previous history of hypertension and diabetes mellitus. The patient was admitted for acute cholecystitis, which was confirmed radiologically by a computed tomography scan with contrast that also showed an incidental right atrial filling defect. A transthoracic echocardiogram showed a 20 × 22-mm spheric echogenic mass in the right atrium with mild mitral annular calcification. Surgical resection was planned using preoperative coronary angiography, which showed no evidence of coronary artery disease. She underwent surgical resection through a right atriotomy and a cardiopulmonary bypass without any adverse events. Grossly, the tumor was a 4 × 3-cm, cauliflower-shaped, purple-colored mass attached to the interatrial septum (Figure 1). A bovine pericardial patch was used to repair the resultant defect. Histopathology showed numerous papillae with endocardial lining and cores containing myocytes as well as scattered elastic fibers (Figure 2). The postoperative period was complicated by an atypical atrial flutter that was treated medically.Figure 2A high-power hematoxylin and eosin stain showing the collagenous cores lined by endothelial cells.View Large Image Figure ViewerDownload Hi-res image Download (PPT) An autopsy series showed an estimated incidence of 0.0017% to 0.33% of primary cardiac tumors; cardiac papillary fibroelastomas (CPFEs) represented only 8%, making this diagnosis extremely rare.1Klarich K.W. Enriquez-Sarano M. Gura G.M. Edwards W.D. Tajik A.J. Seward J.B. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation.J Am Coll Cardiol. 1997; 30: 784-790Abstract Full Text Full Text PDF PubMed Scopus (268) Google Scholar, 2Richard R.W. Tumours of the heart. Review of the subject and report of one hundred fifty cases.Arch Pathol. 1951; 51: 98-128Google Scholar CPFEs represents 80% of all valvular cardiac tumors.3Gowda R.M. Khan I.A. Nair C.K. Mehta N.J. Vasavada B.C. Sacchi T.J. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.Am Heart J. 2003; 146: 404-410Abstract Full Text Full Text PDF PubMed Scopus (486) Google Scholar Valvular CPFE represents 73% of reported cases of CPFE. Most nonvalvular CPFEs originate in the left ventricle, with only a few cases to our knowledge reported in the right atrium, as in our patient (Table 1).4Schiller A.L. Schantz A. Papillary endocardial excrescence of the right atrium: report of two cases.Am J Clin Pathol. 1970; 53: 617-621Crossref PubMed Scopus (22) Google Scholar, 5Schwinger M.E. Katz E. Rotterdam H. Slater J. Weiss E.C. Kronzon I. Right atrial papillary fibroelastoma: diagnosis by transthoracic and transesophageal echocardiography and percutaneous transvenous biopsy.Am Heart J. 1989; 118: 1047-1050Abstract Full Text PDF PubMed Scopus (34) Google Scholar, 6Wasdahl D.A. Wasdahl W.A. Edwards W.D. Fibroelastic papilloma arising in a Chiari network.Clin Cardiol. 1992; 15: 45-47Crossref PubMed Scopus (18) Google Scholar, 7Gallas M.T. Reardon M.J. Reardon P.R. De Felice C.A. Raizner A.E. Mody D.R. Papillary fibroelastoma. A right atrial presentation.Tex Heart Inst J. 1993; 20: 293-295PubMed Google Scholar, 8Crestanello J.A. Orszulak T.A. Giant papillary fibroelastoma of the right atrium: an unusual presentation.Ann Thorac Surg. 2002; 74: 1252-1254Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar, 9Singh H. Alam M. Schwartz D. Martin J.R. Right atrial papillary elastoma: an uncommon location.Echocardiography. 2004; 21: 273-274Crossref PubMed Scopus (6) Google Scholar, 10Hindupur S. Schwabe J.L. Papillary fibroelastoma of the right atrium: an atypical presentation. A case report and review of the literature.J Cardiovasc Surg. 2005; 46: 589-591PubMed Google Scholar, 11Nagano H. Kawahito K. Kobinata T. Nakatani K. Osawa S. Adachi S. et al.Papillary fibroelastoma of the right atrium; report of a case.Kyobu Geka. 2005; 58 ([in Japanese]): 1003-1005PubMed Google Scholar, 12Gabbieri D. Rossi G. Barutti L. Corghi F. Zaca F. Sarandria D. et al.Papillary fibroelastoma of the right atrium as an unusual source of recurrent pulmonary embolism.J Cardiovasc Med. 2006; 7: 373-378Crossref Scopus (11) Google Scholar, 13Lotto A.A. Earl U.M. Owens W.A. Right atrial mass: thrombus, myxoma, or cardiac papillary fibroelastoma?.J Thorac Cardiovasc Surg. 2006; 132: 159-160Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 14Lauten A. Strauch J.T. Wipperman J. Walers T. A rare type of right tumor in a 66-year-old woman.J Thorac Cardiovasc Surg. 2007; 133: 251-252Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar, 15Kim R.W. Jeffery M.E. Smith M.J. Rl Wilensky Woo E.Y. Woo Y.J. Minimally invasive resection of papillary fibroelastoma in a high-risk patient.J Cardiovasc Med (Hagerstown). 2007; 8: 639-641Crossref PubMed Scopus (8) Google Scholar, 16Latif F. Peyton M. Laszik Z. Sivaram C.A. Infective endocarditis of a papillary fibroelastoma on Chiari network of right atrium: a case report.J Am Soc Echocardiography. 2008; 21 (e3-4): 188Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar, 17Abad C. De la Rosa P. Right atrial papillary fibroelastoma associated with atrial septal defect, persistent superior vena cava, and coronary artery disease.J Thorac Cardiovasc Surg. 2008; 136: 538Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar, 18Maybury R. Mullenix P. Greenberg M. Liu M. Trachiotis G. Rare anatomic location of a papillary fibroelastoma.Ann Thorac Surg. 2009; 88: 2034-2035Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar, 19Nwiloh J. Hernandez E. Mercado A. Right atrial papillary fibroelastoma.J Card Surg. 2011; 26: 39-41Crossref PubMed Scopus (3) Google Scholar, 20Marboeuf P. Wautot F. Garot J. Juthier F. Vincentelli A. Ennezat P.V. Maréchaux S. Echocardiographic, cardiac magnetic resonance imaging, surgical and pathological findings of an unusual right atrial tumor: a giant papillary fibroelastoma.Ann Thorac Surg. 2012; 93: e133Abstract Full Text Full Text PDF PubMed Scopus (5) Google ScholarTable 1Case reports of nonvalvular right atrial papillary fibroelastomaStudyYearAge, ySexSymptomSize, mmSchiller et al4Schiller A.L. Schantz A. Papillary endocardial excrescence of the right atrium: report of two cases.Am J Clin Pathol. 1970; 53: 617-621Crossref PubMed Scopus (22) Google Scholar∗This study reported 2 cases.1970N/AN/AN/AN/ASchwinger et al5Schwinger M.E. Katz E. Rotterdam H. Slater J. Weiss E.C. Kronzon I. Right atrial papillary fibroelastoma: diagnosis by transthoracic and transesophageal echocardiography and percutaneous transvenous biopsy.Am Heart J. 1989; 118: 1047-1050Abstract Full Text PDF PubMed Scopus (34) Google Scholar1989N/AN/AN/AN/AWasdahl et al6Wasdahl D.A. Wasdahl W.A. Edwards W.D. Fibroelastic papilloma arising in a Chiari network.Clin Cardiol. 1992; 15: 45-47Crossref PubMed Scopus (18) Google Scholar199285MaleN/AN/AGallas et al7Gallas M.T. Reardon M.J. Reardon P.R. De Felice C.A. Raizner A.E. Mody D.R. Papillary fibroelastoma. A right atrial presentation.Tex Heart Inst J. 1993; 20: 293-295PubMed Google Scholar199369MaleIncidental25 × 20Crestanello et al8Crestanello J.A. Orszulak T.A. Giant papillary fibroelastoma of the right atrium: an unusual presentation.Ann Thorac Surg. 2002; 74: 1252-1254Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar200286FemaleSyncope40Singh et al9Singh H. Alam M. Schwartz D. Martin J.R. Right atrial papillary elastoma: an uncommon location.Echocardiography. 2004; 21: 273-274Crossref PubMed Scopus (6) Google Scholar200475MaleMurmur25 × 25Hindupur et al10Hindupur S. Schwabe J.L. Papillary fibroelastoma of the right atrium: an atypical presentation. A case report and review of the literature.J Cardiovasc Surg. 2005; 46: 589-591PubMed Google Scholar2005N/AN/AN/AN/ANagano et al11Nagano H. Kawahito K. Kobinata T. Nakatani K. Osawa S. Adachi S. et al.Papillary fibroelastoma of the right atrium; report of a case.Kyobu Geka. 2005; 58 ([in Japanese]): 1003-1005PubMed Google Scholar200568FemaleIncidental20Gabbieri et al12Gabbieri D. Rossi G. Barutti L. Corghi F. Zaca F. Sarandria D. et al.Papillary fibroelastoma of the right atrium as an unusual source of recurrent pulmonary embolism.J Cardiovasc Med. 2006; 7: 373-378Crossref Scopus (11) Google Scholar200670FemaleRecurrent PE10 × 10Lotto et al13Lotto A.A. Earl U.M. Owens W.A. Right atrial mass: thrombus, myxoma, or cardiac papillary fibroelastoma?.J Thorac Cardiovasc Surg. 2006; 132: 159-160Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar200660FemaleW/U of CAD20 × 22Lauten et al14Lauten A. Strauch J.T. Wipperman J. Walers T. A rare type of right tumor in a 66-year-old woman.J Thorac Cardiovasc Surg. 2007; 133: 251-252Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar200766FemaleIncidental28 × 25Kim et al15Kim R.W. Jeffery M.E. Smith M.J. Rl Wilensky Woo E.Y. Woo Y.J. Minimally invasive resection of papillary fibroelastoma in a high-risk patient.J Cardiovasc Med (Hagerstown). 2007; 8: 639-641Crossref PubMed Scopus (8) Google Scholar200762FemaleIncidentalN/ALatif et al16Latif F. Peyton M. Laszik Z. Sivaram C.A. Infective endocarditis of a papillary fibroelastoma on Chiari network of right atrium: a case report.J Am Soc Echocardiography. 2008; 21 (e3-4): 188Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar200841MaleIEC12 × 20Abad et al17Abad C. De la Rosa P. Right atrial papillary fibroelastoma associated with atrial septal defect, persistent superior vena cava, and coronary artery disease.J Thorac Cardiovasc Surg. 2008; 136: 538Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar200860MaleW/U of TIA20 × 15Maybury et al18Maybury R. Mullenix P. Greenberg M. Liu M. Trachiotis G. Rare anatomic location of a papillary fibroelastoma.Ann Thorac Surg. 2009; 88: 2034-2035Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar200974MalePresyncope10 × 10Nwiloh et al19Nwiloh J. Hernandez E. Mercado A. Right atrial papillary fibroelastoma.J Card Surg. 2011; 26: 39-41Crossref PubMed Scopus (3) Google Scholar201173MaleW/U of TIA33 × 30Marboeuf et al20Marboeuf P. Wautot F. Garot J. Juthier F. Vincentelli A. Ennezat P.V. Maréchaux S. Echocardiographic, cardiac magnetic resonance imaging, surgical and pathological findings of an unusual right atrial tumor: a giant papillary fibroelastoma.Ann Thorac Surg. 2012; 93: e133Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar201265MalePE30 × 28N/A, Not available; W/U, work up; PE, pulmonary embolism; CAD, coronary artery disease; IEC, infective endocarditis; TIA, transient ischemic attack.∗ This study reported 2 cases. Open table in a new tab N/A, Not available; W/U, work up; PE, pulmonary embolism; CAD, coronary artery disease; IEC, infective endocarditis; TIA, transient ischemic attack. On reviewing the literature on nonvalvular right atrial CPFEs (Table 1), most cases were discovered incidentally during a cardiac work-up for unrelated symptoms or signs including transient ischemic attacks, syncope, or murmurs. Other cases were linked to recurrent pulmonary embolism. One case presented as recurrent Staphylococcus aureus bacteremia secondary to infected CPFEs growing on the right atrial Chiari formation.16Latif F. Peyton M. Laszik Z. Sivaram C.A. Infective endocarditis of a papillary fibroelastoma on Chiari network of right atrium: a case report.J Am Soc Echocardiography. 2008; 21 (e3-4): 188Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Right-sided CPFEs can present as a pulmonary embolism in 0.4% of all cases of CPFEs.3Gowda R.M. Khan I.A. Nair C.K. Mehta N.J. Vasavada B.C. Sacchi T.J. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.Am Heart J. 2003; 146: 404-410Abstract Full Text Full Text PDF PubMed Scopus (486) Google Scholar Physical examination of the patient might be significant for positional systolic and diastolic murmurs that can occur depending on the size, mobility, and location of the tumor. The diastolic tumor murmur was described in 1 case of CPFE to involve the tricuspid valve compared with 30% of cases of atrial myxomas.21Frumin H. O’Donnell L. Kerin N.Z. Levine F. Nathan Jr., L.E. Klein S.P. Two-dimensional echo- cardiographic detection and diagnostic features of tricuspid papillary fibroelastoma.J Am Coll Cardiol. 1983; 2: 1016-1018Abstract Full Text PDF PubMed Scopus (35) Google Scholar Our review showed 1 case of right-sided nonvalvular CPFE discovered during the work-up of an apical murmur.9Singh H. Alam M. Schwartz D. Martin J.R. Right atrial papillary elastoma: an uncommon location.Echocardiography. 2004; 21: 273-274Crossref PubMed Scopus (6) Google Scholar Transthoracic echocardiography, with an estimated sensitivity and specificity of 88.9% and 87.8%, respectively, remains the most common initial test to detect CPFEs. For tumors smaller than 20 mm, the reported transthoracic echocardiography sensitivity decreases to 61.9% compared with the more sensitive transesophageal echocardiography, which has a sensitivity of 76.6%.22Sun J.P. Asher C.R. Yang X.S. Cheng G.G. Scalia G.M. Massed A.G. et al.Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients.Circulation. 2001; 103: 2687-2693Crossref PubMed Scopus (355) Google Scholar Good correlation between echocardiography and pathologic size of the tumor has been reported (r = 0.87; P < .001). Few cases of CPFE have been detected with computed tomography, as in our patient.15Kim R.W. Jeffery M.E. Smith M.J. Rl Wilensky Woo E.Y. Woo Y.J. Minimally invasive resection of papillary fibroelastoma in a high-risk patient.J Cardiovasc Med (Hagerstown). 2007; 8: 639-641Crossref PubMed Scopus (8) Google Scholar Gadolinium-enhanced magnetic resonance imaging with enhancement of the periphery of the tumor secondary to trapping of the gadolinium in the fronds of collagen and elastin also has been described.23Atalay M.K. Taner A.T. Gradual enhancement of a large left atrial papillary fibroelastoma on cardiac magnetic resonance: the waiting game.Tex Heart Inst J. 2010; 37: 612-613PubMed Google Scholar Histopathology shows an avascular core of elastin and immature collagen fibers with a single layer of endocardium outlining the papillomatous-like tumor.24Burke A. Virmani R. Papillary fibroelastoma: tumors of the heart and great vessels.AFIP Atlas of Tumor Pathology. 1996; 16: 47-54Google Scholar After placing the tumor in water, sea anemone–like branches with multiple papillary fronds arising from a central stalk create its characteristic gross appearance.25Kim K.H. Choi J.B. Papillary fibroelastoma in tricuspid valve: an unusual cause of atypical chest pain.J Thorac Cardiovasc Surg. 2013; 145: 1131Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar In our patient we were able to identify numerous myocytes in the core of the papillomatous projections, which rarely has been reported. All reported cases of right-sided CPFE have been managed with surgical resection with cardiopulmonary bypass except for 1 case managed through a minimally invasive right thoracotomy.15Kim R.W. Jeffery M.E. Smith M.J. Rl Wilensky Woo E.Y. Woo Y.J. Minimally invasive resection of papillary fibroelastoma in a high-risk patient.J Cardiovasc Med (Hagerstown). 2007; 8: 639-641Crossref PubMed Scopus (8) Google Scholar Three cases had a concomitant coronary artery bypass graft with surgical resection of the right-sided CPFE. Recurrence after surgical resection has not been described in the literature even with prolonged follow-up periods of up to 114 months.17Abad C. De la Rosa P. Right atrial papillary fibroelastoma associated with atrial septal defect, persistent superior vena cava, and coronary artery disease.J Thorac Cardiovasc Surg. 2008; 136: 538Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar