Journal of Cardiology Cases 9 (2014) 8–10
Contents lists available at www.sciencedirect.com
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
A rare nonvalvular left ventricular papillary fibroelastoma: A case report Forum Kamdar (MD) a , Sithu Win (MD) a , J. Carlos Manivel (MD) b , Sara Shumway (MD) c , Emil Missov (MD, PhD) a,∗ a
Division of Cardiology, University of Minnesota Medical Center, 420 Delaware Street SE, Minneapolis, MN 55455, USA Department of Laboratory Medicine and Pathology, University of Minnesota Medical Center, Minneapolis, MN 55455, USA c Division of Cardiothoracic Surgery, University of Minnesota Medical Center, Minneapolis, MN 55455, USA b
a r t i c l e
i n f o
Article history: Received 8 July 2013 Received in revised form 25 July 2013 Accepted 21 August 2013 Keywords: Papillary fibroelastoma Nonvalvular Transesophageal echocardiography Cardiac magnetic resonance imaging
a b s t r a c t Papillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. We report the case of a 66-year-old Caucasian male with acute bilateral basal ganglia infarctions found to have a mass adherent to the left ventricular septum by transesophageal echocardiography. The mass was identified as a rare nonvalvular cardiac papillary fibroelastoma based on echogenicity, pedunculated nature, and typical motion. Tissue characterization by cardiac magnetic resonance imaging demonstrated homogeneously hypo-intense signal on T2 weighted imaging and signal hyperintensity after administration of gadolinium contrast, confirming the fibroelastic nature of the mass. Surgical excision was performed via ventriculotomy and histopathologic examination was pathognomonic of a papillary fibroelastoma. We conclude that transesophageal echocardiography provides high diagnostic certainty in patients with cardiac papillary fibroelastomas and can reliably identify atypical locations of these tumors on nonvalvular surfaces. A multimodality imaging approach is not necessarily indicated in all patients with this condition. © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Introduction Primary cardiac tumors are rare with a lifetime incidence of 0.0017–0.02% [1]. Papillary fibroelastomas are the third most common cardiac tumors after myxomas and lipomas and account for less than 10% of all primary cardiac tumors. The valvular endocardium is the preferential attachment site 80% of the time; nonvalvular manifestations remain exceptionally rare [2,3]. Whether a multimodality imaging approach offers significant added value compared with echocardiography alone in the diagnostic evaluation of cardiac papillary fibroelastomas is controversial. We illustrate this point with the case of a rare nonvalvular left ventricular papillary fibroelastoma. Case report A 66-year-old Caucasian male with acute onset of memory difficulties was found to have large bilateral basal ganglia
∗ Corresponding author. Tel.: +1 612 625 7924; fax: +1 612 624 4411. E-mail address: [email protected] (E. Missov).
infarctions without evidence of cerebrovascular or carotid artery disease on magnetic resonance angiography. A screening transthoracic echocardiogram was unremarkable. However, transesophageal echocardiography (TEE) demonstrated a highly mobile, 0.9 cm × 0.9 cm, spherical, homogenous echo-dense mass adherent to the left ventricular septal wall. Based on its characteristic appearance and echogenicity, pedunculated nature, and mobility, the mass was identified as a papillary fibroelastoma (Fig. 1A and B; Supplementary data: Videos 1 and 2). Cardiac magnetic resonance (CMR) imaging was performed for further tissue characterization. The mass appeared homogeneously hypo-intense on T2 weighted imaging. Delayed enhancement images after administration of gadolinium demonstrated signal hyperintensity caused by the fibroelastic nature of the mass (Fig. 1C and D). Supplementary data associated with this artican be found, in the online version, at cle http://dx.doi.org/10.1016/j.jccase.2013.08.012. The patient underwent excision of the mass via ventriculotomy. The surgical specimen was a friable, gelatinous mass that was loosely attached to the left ventricular septal wall (Fig. 2A). Cardiac papillary fibroelastoma was confirmed by histopathology. Low
1878-5409/$ – see front matter © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jccase.2013.08.012
F. Kamdar et al. / Journal of Cardiology Cases 9 (2014) 8–10
9
Fig. 1. Transesophageal echocardiography and cardiac magnetic resonance imaging: (A) transesophageal echocardiogram in the left ventricular outflow tract view showing a spherical, homogenous, echo-dense mass measuring approximately 0.9 cm × 0.9 cm arising from the left ventricular septum (arrow), (B) magnified view of the left ventricular septal mass, (C) cardiac magnetic resonance delayed enhancement images in the long axis and (D) short axis showing signal hyperintensity of left ventricular septal mass after gadolinium administration (arrow).
Fig. 2. Surgical pathology and histopathology: (A) surgical specimen of gelatinous, friable, papillary mass resected from the left ventricular septum via ventriculotomy, (B) low power hematoxylin and eosin photomicrograph demonstrating branching papillae consisting of hypocellular collagenous cores lined by a layer of endocardial cells and loose myxoid matrix, (C) immunoperoxidase stain for CD34 highlights the endocardial lining and (D) trichrome stain highlights the collagenous nature of the cores.
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F. Kamdar et al. / Journal of Cardiology Cases 9 (2014) 8–10
magnification hematoxylin and eosin stain demonstrated branching papillae consisting of hypocellular collagenous cores lined by a layer of endocardial cells and loose myxoid matrix (Fig. 2B). Immunoperoxidase stain for CD34 highlighted the endocardial lining (Fig. 2C); trichrome stain demonstrated the collagenous nature of the cores (Fig. 2D). Discussion Cardiac papillary fibroelastomas are histologically benign slow-growing tumors plagued with significant clinical complications. They carry a high embolic potential given the friability of their tissue matrix. The mode of clinical presentation is a transient ischemic attack or a cerebrovascular accident in 53% of patients with symptomatic fibroelastomas [2,3]. Surgical resection is curative and can prevent occurrence or reoccurrence of embolic phenomena. From a histopathology viewpoint, cardiac papillary fibroelastomas may be considered reactive tumoral lesions or organized mural thrombi rather than true neoplasms. This is supported by the fact that they are encountered frequently after cardiac surgery [4–6]. The reported case of a cardiac papillary fibroelastoma originating from the left ventricular aspect of the distal interventricular septum is rare. The incidence of nonvalvular left ventricular fibroelastomas was only 9% in the series reported by Gowda et al. [2]. The TEE images obtained were of high diagnostic quality, identifying a cardiac papillary fibroelastoma with a high degree of clinical certainty. CMR was utilized only to confirm the diagnosis and for additional tissue characterization of the mass. There was a high degree of concordance between TEE and CMR, both techniques accurately identifying the location of the papillary fibroelastoma, confirmed at the time of surgery.
Conclusions The case emphasizes the important role and high diagnostic certainty of TEE in identifying potential cardiac sources of emboli, including cardiac papillary fibroelastomas in patients with cerebrovascular events. TEE can reliably identify rare and atypical locations of these tumors on nonvalvular surfaces and remains essential for surgical planning and intraoperative guidance. Additional high-end imaging modalities may not offer significant added value and may not be necessarily indicated in all patients with this condition [7]. Conflict of interest Authors declare no conflict of interest. References [1] Silverman N. Primary cardiac tumors. Ann Surg 1980;191:127–38. [2] Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003;146:404–10. [3] Bossert T, Gummert JF, Battellini R, Richter M, Barten M, Walther T, Falk V, Wilhelm F. Surgical experience with 77 primary cardiac tumors. Interact CardioVasc Thorac Surg 2005;4:311–5. [4] Kurup AN, Tazelaar HD, EdwardsW.D. Burke AP, Virmani R, Klarich KW, Orszulak TA. Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990 to 2000). Hum Pathol 2002;33:1165–9. [5] Howard RA, Aldea GS, Shapira OM, Kaznica JM, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 2000;70:335–6. [6] Kumar TK, Kuehl K, Reyes C, Talwar S, Moulick A, Jonas RA. Multiple papillary fibroelastomas of the heart. Ann Thorac Surg 2009;88:e66–7. [7] Basoglu A, De Wolf A, Dendale P, Vanbockrijck M, Hendrikx M, Vranckx P. Echocardiography remains the standard for the diagnostic evaluation of left ventricular tumors: a case report with anatomical correlation. Eur J Echocardiogr 2007;8:401–3.
Contents lists available at www.sciencedirect.com
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
A rare nonvalvular left ventricular papillary fibroelastoma: A case report Forum Kamdar (MD) a , Sithu Win (MD) a , J. Carlos Manivel (MD) b , Sara Shumway (MD) c , Emil Missov (MD, PhD) a,∗ a
Division of Cardiology, University of Minnesota Medical Center, 420 Delaware Street SE, Minneapolis, MN 55455, USA Department of Laboratory Medicine and Pathology, University of Minnesota Medical Center, Minneapolis, MN 55455, USA c Division of Cardiothoracic Surgery, University of Minnesota Medical Center, Minneapolis, MN 55455, USA b
a r t i c l e
i n f o
Article history: Received 8 July 2013 Received in revised form 25 July 2013 Accepted 21 August 2013 Keywords: Papillary fibroelastoma Nonvalvular Transesophageal echocardiography Cardiac magnetic resonance imaging
a b s t r a c t Papillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. We report the case of a 66-year-old Caucasian male with acute bilateral basal ganglia infarctions found to have a mass adherent to the left ventricular septum by transesophageal echocardiography. The mass was identified as a rare nonvalvular cardiac papillary fibroelastoma based on echogenicity, pedunculated nature, and typical motion. Tissue characterization by cardiac magnetic resonance imaging demonstrated homogeneously hypo-intense signal on T2 weighted imaging and signal hyperintensity after administration of gadolinium contrast, confirming the fibroelastic nature of the mass. Surgical excision was performed via ventriculotomy and histopathologic examination was pathognomonic of a papillary fibroelastoma. We conclude that transesophageal echocardiography provides high diagnostic certainty in patients with cardiac papillary fibroelastomas and can reliably identify atypical locations of these tumors on nonvalvular surfaces. A multimodality imaging approach is not necessarily indicated in all patients with this condition. © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Introduction Primary cardiac tumors are rare with a lifetime incidence of 0.0017–0.02% [1]. Papillary fibroelastomas are the third most common cardiac tumors after myxomas and lipomas and account for less than 10% of all primary cardiac tumors. The valvular endocardium is the preferential attachment site 80% of the time; nonvalvular manifestations remain exceptionally rare [2,3]. Whether a multimodality imaging approach offers significant added value compared with echocardiography alone in the diagnostic evaluation of cardiac papillary fibroelastomas is controversial. We illustrate this point with the case of a rare nonvalvular left ventricular papillary fibroelastoma. Case report A 66-year-old Caucasian male with acute onset of memory difficulties was found to have large bilateral basal ganglia
∗ Corresponding author. Tel.: +1 612 625 7924; fax: +1 612 624 4411. E-mail address: [email protected] (E. Missov).
infarctions without evidence of cerebrovascular or carotid artery disease on magnetic resonance angiography. A screening transthoracic echocardiogram was unremarkable. However, transesophageal echocardiography (TEE) demonstrated a highly mobile, 0.9 cm × 0.9 cm, spherical, homogenous echo-dense mass adherent to the left ventricular septal wall. Based on its characteristic appearance and echogenicity, pedunculated nature, and mobility, the mass was identified as a papillary fibroelastoma (Fig. 1A and B; Supplementary data: Videos 1 and 2). Cardiac magnetic resonance (CMR) imaging was performed for further tissue characterization. The mass appeared homogeneously hypo-intense on T2 weighted imaging. Delayed enhancement images after administration of gadolinium demonstrated signal hyperintensity caused by the fibroelastic nature of the mass (Fig. 1C and D). Supplementary data associated with this artican be found, in the online version, at cle http://dx.doi.org/10.1016/j.jccase.2013.08.012. The patient underwent excision of the mass via ventriculotomy. The surgical specimen was a friable, gelatinous mass that was loosely attached to the left ventricular septal wall (Fig. 2A). Cardiac papillary fibroelastoma was confirmed by histopathology. Low
1878-5409/$ – see front matter © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jccase.2013.08.012
F. Kamdar et al. / Journal of Cardiology Cases 9 (2014) 8–10
9
Fig. 1. Transesophageal echocardiography and cardiac magnetic resonance imaging: (A) transesophageal echocardiogram in the left ventricular outflow tract view showing a spherical, homogenous, echo-dense mass measuring approximately 0.9 cm × 0.9 cm arising from the left ventricular septum (arrow), (B) magnified view of the left ventricular septal mass, (C) cardiac magnetic resonance delayed enhancement images in the long axis and (D) short axis showing signal hyperintensity of left ventricular septal mass after gadolinium administration (arrow).
Fig. 2. Surgical pathology and histopathology: (A) surgical specimen of gelatinous, friable, papillary mass resected from the left ventricular septum via ventriculotomy, (B) low power hematoxylin and eosin photomicrograph demonstrating branching papillae consisting of hypocellular collagenous cores lined by a layer of endocardial cells and loose myxoid matrix, (C) immunoperoxidase stain for CD34 highlights the endocardial lining and (D) trichrome stain highlights the collagenous nature of the cores.
10
F. Kamdar et al. / Journal of Cardiology Cases 9 (2014) 8–10
magnification hematoxylin and eosin stain demonstrated branching papillae consisting of hypocellular collagenous cores lined by a layer of endocardial cells and loose myxoid matrix (Fig. 2B). Immunoperoxidase stain for CD34 highlighted the endocardial lining (Fig. 2C); trichrome stain demonstrated the collagenous nature of the cores (Fig. 2D). Discussion Cardiac papillary fibroelastomas are histologically benign slow-growing tumors plagued with significant clinical complications. They carry a high embolic potential given the friability of their tissue matrix. The mode of clinical presentation is a transient ischemic attack or a cerebrovascular accident in 53% of patients with symptomatic fibroelastomas [2,3]. Surgical resection is curative and can prevent occurrence or reoccurrence of embolic phenomena. From a histopathology viewpoint, cardiac papillary fibroelastomas may be considered reactive tumoral lesions or organized mural thrombi rather than true neoplasms. This is supported by the fact that they are encountered frequently after cardiac surgery [4–6]. The reported case of a cardiac papillary fibroelastoma originating from the left ventricular aspect of the distal interventricular septum is rare. The incidence of nonvalvular left ventricular fibroelastomas was only 9% in the series reported by Gowda et al. [2]. The TEE images obtained were of high diagnostic quality, identifying a cardiac papillary fibroelastoma with a high degree of clinical certainty. CMR was utilized only to confirm the diagnosis and for additional tissue characterization of the mass. There was a high degree of concordance between TEE and CMR, both techniques accurately identifying the location of the papillary fibroelastoma, confirmed at the time of surgery.
Conclusions The case emphasizes the important role and high diagnostic certainty of TEE in identifying potential cardiac sources of emboli, including cardiac papillary fibroelastomas in patients with cerebrovascular events. TEE can reliably identify rare and atypical locations of these tumors on nonvalvular surfaces and remains essential for surgical planning and intraoperative guidance. Additional high-end imaging modalities may not offer significant added value and may not be necessarily indicated in all patients with this condition [7]. Conflict of interest Authors declare no conflict of interest. References [1] Silverman N. Primary cardiac tumors. Ann Surg 1980;191:127–38. [2] Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003;146:404–10. [3] Bossert T, Gummert JF, Battellini R, Richter M, Barten M, Walther T, Falk V, Wilhelm F. Surgical experience with 77 primary cardiac tumors. Interact CardioVasc Thorac Surg 2005;4:311–5. [4] Kurup AN, Tazelaar HD, EdwardsW.D. Burke AP, Virmani R, Klarich KW, Orszulak TA. Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990 to 2000). Hum Pathol 2002;33:1165–9. [5] Howard RA, Aldea GS, Shapira OM, Kaznica JM, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 2000;70:335–6. [6] Kumar TK, Kuehl K, Reyes C, Talwar S, Moulick A, Jonas RA. Multiple papillary fibroelastomas of the heart. Ann Thorac Surg 2009;88:e66–7. [7] Basoglu A, De Wolf A, Dendale P, Vanbockrijck M, Hendrikx M, Vranckx P. Echocardiography remains the standard for the diagnostic evaluation of left ventricular tumors: a case report with anatomical correlation. Eur J Echocardiogr 2007;8:401–3.
