Case Study
Unique presentation of a primary pleomorphic sarcoma of the left atrium
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(8) 973–975 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314529782 aan.sagepub.com
Alexis Lake1, Omer Ashraf2, Rachel Maywald3 and Homayoun Jalali1
Abstract This report describes an extremely rare cause of a common presentation. A 59-year-old man who presented with symptoms of progressive cardiac failure, was demonstrated to have a large left intraatrial mass on computed tomography pulmonary angiography and an echocardiogram. He proceeded to urgent surgery under cardiopulmonary bypass. Histology of the resected mass diagnosed primary pleomorphic sarcoma of the left atrium. This exceedingly rare malignancy has a poor prognosis and often presents with nonspecific symptoms. Echocardiography remains vital in diagnosis, and treatment is primarily surgical excision, with some reports of adjuvant chemotherapy.
Keywords Echocardiography, heart atria, heart failure, heart neoplasms, sarcoma
Introduction Primary cardiac sarcoma is a malignancy with a uniformly poor prognosis.1 Its scarcity is emphasized in the literature, with an incidence of 0.001%–0.03% in an autopsy series.2 For this reason, cardiac sarcoma is an extremely rare cause of heart failure and presents an interesting diagnostic and treatment quandary.
Case report A 59-year-old man presented with a 2-week history of exertional dyspnea on a background of a 1-year history of bilateral amaurosis fugax. A computed tomography pulmonary angiogram revealed a lobulated homogenous low-attenuation mass measuring up to 8 cm within the left atrium, filling the left atrial appendage and extending across the mitral valve into the left ventricle (Figure 1). Transthoracic echocardiography confirmed these findings, showing the mass to arise from the posterior atrial wall and prolapse through the mitral valve. There was no mitral regurgitation. The findings were subsequently confirmed by intraoperative transesophageal echocardiography (Figure 2). The patient underwent urgent open resection of the left atrial mass under cardiopulmonary bypass. Intraoperatively, a large multiloculated mass was discovered arising from the medial
part of the left atrium (Figure 3). Despite the tumor being adherent to the posterior leaflet of the mitral valve, there was involvement of a single mitral valve chorda only. This was resected and the mitral valve remained competent. All of the adhesions to the left atrial wall were detached. On histological analysis, the excised tumor portions weighed 81 g and showed a pale myxoid to fibrous cut surface with areas of hemorrhage (Figure 4A). Microscopically, the tumor showed variation in cellularity with epithelioid and spindle cell morphology (Figure 4B). There was moderate pleomorphism with easily identified mitoses and areas of tumor necrosis (Figure 4C, 4D). The architecture was variable with fascicular areas and areas with tumor cells accentuated around compressed vessels. 1 Department of Cardiothoracic Surgery, The Prince Charles Hospital, Brisbane, Queensland, Australia 2 Department of Cardiothoracic Surgery, The Townsville Hospital, Townsville, Queensland, Australia 3 Department of Anatomical Pathology, The Prince Charles Hospital, Brisbane, Queensland, Australia
Corresponding author: Omer Ashraf, The Townsville Hospital, 100 Angus Smith Drive, Douglas, Queensland 4814, Australia. Email: [email protected]
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
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Asian Cardiovascular & Thoracic Annals 23(8)
Figure 1. Sagittal section of a computed tomogram showing a large mass within the left atrium.
The histological diagnosis was pleomorphic sarcoma with smooth muscle and vascular differentiation. Postoperative transthoracic echocardiography revealed mild mitral regurgitation with no further visualization of the mass. The patient was discharged and scheduled for outpatient chemotherapy. Computed tomography and magnetic resonance imaging was performed at 2 months postoperatively, which revealed development of 3 new masses in the left atrium around the site of the previously excised tumor (Figure 5). Repeat surgery was undertaken to resect the new growths. Histopathology confirmed them to be recurrent pleomorphic sarcomas. Postoperative echocardiography revealed normal left ventricular function and no signs of any intracardiac shunt or residual tumor. Unfortunately, the patient developed a second recurrence of his malignancy 4 months after this resection, which required further surgery. A year on, this remarkable gentleman remains alive and is closely monitored for further recurrence.
Discussion
Figure 2. Intraoperative transesophageal echocardiogram showing a large left atrial mass prolapsing across the mitral valve into the left ventricle.
Figure 3. The resected tumor specimen on display after excision during the first surgery.
Cardiac sarcomas present late, and often with nonspecific symptoms of malaise and fatigue.2 A case series by Centofanti and colleagues3 described 5 cases of malignant primary cardiac tumors diagnosed over a 17-year period. Interestingly, only one of these patients presented with palpitations and the others were completely asymptomatic. A review of the literature revealed 3 case reports describing a presentation of pleomorphic sarcoma similar to that of our patient.1,4,5 The overwhelming presenting complaint in these cases was progressive shortness of breath and fatigue, and two cases had infective symptoms of pyrexia, chills, and productive cough. None describe embolic phenomena of transient ischemic attacks or amaurosis fugax, as seen in our case. In a review of cardiac sarcomas, Shanmugam6 describes obstruction, arrhythmogenesis, embolism, and cardiorespiratory failure as 4 general mechanisms producing the symptomatology associated with these cardiac malignancies. Transthoracic echocardiography is an essential tool in diagnosis due to its ability to directly visualize cardiac masses, accurately describe the location, and importantly, evaluate valve function and the hemodynamic effect on the heart.7 While the management of cardiac sarcoma remains primarily surgical, it also includes adjuvant chemotherapy and radiation; however, the rarity of this disease means that published data on treatment outcomes is sparse. On histology, sarcomas represent the most common type of primary cardiac malignancy, with angiosarcoma being the most common subtype, followed by rhabdomyosarcoma.8 The tumor in our patient was diagnosed as
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
Lake et al.
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Figure 4. Hematoxylin and eosin stained sections of the tumor showing: (A) fibrous areas (original magnification 20); (B) areas of variable cellularity, with both hypercellular and hypocellular areas (original magnification 20); (C) areas of tumor necrosis throughout the specimen (original magnification 40); and (D) moderate pleomorphism within the tumor cells (original magnification 400).
Conflicts of interest statement None declared.
References
Figure 5. Follow-up magnetic resonance imaging after the first surgery, showing recurrence of tumor growths within the left atrium.
pleomorphic sarcoma with vascular and smooth muscle differentiation. As demonstrated in our report, this malignancy is often locally recurrent. The long-term outcome of cardiac sarcoma is poor, and the sheer rarity of these cancers means there remains no united approach to their management. Funding
1. Hsieh CH, Seak CJ, Chiu TF, Chen JC and Li CH. An uncommon cause of heart failure: cardiac sarcomas in the left atrium. J Emerg Med 2011; 40: 123–124. 2. Hamidi M, Moody JS, Weigel TL and Kozak KR. Primary cardiac sarcoma. Ann Thorac Surg 2010; 90: 176–181. 3. Centofanti P, Di Rosa E, Deorsola L, et al. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999; 68: 1236–1241. 4. Suh SH, Park TH, Yoo JN, et al. Primary undifferentiated pleomorphic sarcoma of the left atrium that presented as acute pulmonary edema. Yonsei Med J 2007; 48: 131–134. 5. Patil HR, Singh D and Hajdu M. Cardiac sarcoma presenting as heart failure and diagnosed as recurrent myxoma by echocardiogram. Eur J Echocardiogr 2010; 11: E12. 6. Shanmugam G. Primary cardiac sarcoma. Eur J Cardiothorac Surg 2006; 29: 925–932. 7. Ekmektzoglou KA, Samelis GF and Xanthos T. Heart and tumors: location, metastasis, clinical manifestations, diagnostic approaches and therapeutic considerations. J Cardiovasc Med (Hagerstown) 2008; 9: 769–777. 8. Shapiro LM. Cardiac tumours: diagnosis and management [Review]. Heart 2001; 85: 218–222.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
Unique presentation of a primary pleomorphic sarcoma of the left atrium
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(8) 973–975 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314529782 aan.sagepub.com
Alexis Lake1, Omer Ashraf2, Rachel Maywald3 and Homayoun Jalali1
Abstract This report describes an extremely rare cause of a common presentation. A 59-year-old man who presented with symptoms of progressive cardiac failure, was demonstrated to have a large left intraatrial mass on computed tomography pulmonary angiography and an echocardiogram. He proceeded to urgent surgery under cardiopulmonary bypass. Histology of the resected mass diagnosed primary pleomorphic sarcoma of the left atrium. This exceedingly rare malignancy has a poor prognosis and often presents with nonspecific symptoms. Echocardiography remains vital in diagnosis, and treatment is primarily surgical excision, with some reports of adjuvant chemotherapy.
Keywords Echocardiography, heart atria, heart failure, heart neoplasms, sarcoma
Introduction Primary cardiac sarcoma is a malignancy with a uniformly poor prognosis.1 Its scarcity is emphasized in the literature, with an incidence of 0.001%–0.03% in an autopsy series.2 For this reason, cardiac sarcoma is an extremely rare cause of heart failure and presents an interesting diagnostic and treatment quandary.
Case report A 59-year-old man presented with a 2-week history of exertional dyspnea on a background of a 1-year history of bilateral amaurosis fugax. A computed tomography pulmonary angiogram revealed a lobulated homogenous low-attenuation mass measuring up to 8 cm within the left atrium, filling the left atrial appendage and extending across the mitral valve into the left ventricle (Figure 1). Transthoracic echocardiography confirmed these findings, showing the mass to arise from the posterior atrial wall and prolapse through the mitral valve. There was no mitral regurgitation. The findings were subsequently confirmed by intraoperative transesophageal echocardiography (Figure 2). The patient underwent urgent open resection of the left atrial mass under cardiopulmonary bypass. Intraoperatively, a large multiloculated mass was discovered arising from the medial
part of the left atrium (Figure 3). Despite the tumor being adherent to the posterior leaflet of the mitral valve, there was involvement of a single mitral valve chorda only. This was resected and the mitral valve remained competent. All of the adhesions to the left atrial wall were detached. On histological analysis, the excised tumor portions weighed 81 g and showed a pale myxoid to fibrous cut surface with areas of hemorrhage (Figure 4A). Microscopically, the tumor showed variation in cellularity with epithelioid and spindle cell morphology (Figure 4B). There was moderate pleomorphism with easily identified mitoses and areas of tumor necrosis (Figure 4C, 4D). The architecture was variable with fascicular areas and areas with tumor cells accentuated around compressed vessels. 1 Department of Cardiothoracic Surgery, The Prince Charles Hospital, Brisbane, Queensland, Australia 2 Department of Cardiothoracic Surgery, The Townsville Hospital, Townsville, Queensland, Australia 3 Department of Anatomical Pathology, The Prince Charles Hospital, Brisbane, Queensland, Australia
Corresponding author: Omer Ashraf, The Townsville Hospital, 100 Angus Smith Drive, Douglas, Queensland 4814, Australia. Email: [email protected]
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
974
Asian Cardiovascular & Thoracic Annals 23(8)
Figure 1. Sagittal section of a computed tomogram showing a large mass within the left atrium.
The histological diagnosis was pleomorphic sarcoma with smooth muscle and vascular differentiation. Postoperative transthoracic echocardiography revealed mild mitral regurgitation with no further visualization of the mass. The patient was discharged and scheduled for outpatient chemotherapy. Computed tomography and magnetic resonance imaging was performed at 2 months postoperatively, which revealed development of 3 new masses in the left atrium around the site of the previously excised tumor (Figure 5). Repeat surgery was undertaken to resect the new growths. Histopathology confirmed them to be recurrent pleomorphic sarcomas. Postoperative echocardiography revealed normal left ventricular function and no signs of any intracardiac shunt or residual tumor. Unfortunately, the patient developed a second recurrence of his malignancy 4 months after this resection, which required further surgery. A year on, this remarkable gentleman remains alive and is closely monitored for further recurrence.
Discussion
Figure 2. Intraoperative transesophageal echocardiogram showing a large left atrial mass prolapsing across the mitral valve into the left ventricle.
Figure 3. The resected tumor specimen on display after excision during the first surgery.
Cardiac sarcomas present late, and often with nonspecific symptoms of malaise and fatigue.2 A case series by Centofanti and colleagues3 described 5 cases of malignant primary cardiac tumors diagnosed over a 17-year period. Interestingly, only one of these patients presented with palpitations and the others were completely asymptomatic. A review of the literature revealed 3 case reports describing a presentation of pleomorphic sarcoma similar to that of our patient.1,4,5 The overwhelming presenting complaint in these cases was progressive shortness of breath and fatigue, and two cases had infective symptoms of pyrexia, chills, and productive cough. None describe embolic phenomena of transient ischemic attacks or amaurosis fugax, as seen in our case. In a review of cardiac sarcomas, Shanmugam6 describes obstruction, arrhythmogenesis, embolism, and cardiorespiratory failure as 4 general mechanisms producing the symptomatology associated with these cardiac malignancies. Transthoracic echocardiography is an essential tool in diagnosis due to its ability to directly visualize cardiac masses, accurately describe the location, and importantly, evaluate valve function and the hemodynamic effect on the heart.7 While the management of cardiac sarcoma remains primarily surgical, it also includes adjuvant chemotherapy and radiation; however, the rarity of this disease means that published data on treatment outcomes is sparse. On histology, sarcomas represent the most common type of primary cardiac malignancy, with angiosarcoma being the most common subtype, followed by rhabdomyosarcoma.8 The tumor in our patient was diagnosed as
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
Lake et al.
975
Figure 4. Hematoxylin and eosin stained sections of the tumor showing: (A) fibrous areas (original magnification 20); (B) areas of variable cellularity, with both hypercellular and hypocellular areas (original magnification 20); (C) areas of tumor necrosis throughout the specimen (original magnification 40); and (D) moderate pleomorphism within the tumor cells (original magnification 400).
Conflicts of interest statement None declared.
References
Figure 5. Follow-up magnetic resonance imaging after the first surgery, showing recurrence of tumor growths within the left atrium.
pleomorphic sarcoma with vascular and smooth muscle differentiation. As demonstrated in our report, this malignancy is often locally recurrent. The long-term outcome of cardiac sarcoma is poor, and the sheer rarity of these cancers means there remains no united approach to their management. Funding
1. Hsieh CH, Seak CJ, Chiu TF, Chen JC and Li CH. An uncommon cause of heart failure: cardiac sarcomas in the left atrium. J Emerg Med 2011; 40: 123–124. 2. Hamidi M, Moody JS, Weigel TL and Kozak KR. Primary cardiac sarcoma. Ann Thorac Surg 2010; 90: 176–181. 3. Centofanti P, Di Rosa E, Deorsola L, et al. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999; 68: 1236–1241. 4. Suh SH, Park TH, Yoo JN, et al. Primary undifferentiated pleomorphic sarcoma of the left atrium that presented as acute pulmonary edema. Yonsei Med J 2007; 48: 131–134. 5. Patil HR, Singh D and Hajdu M. Cardiac sarcoma presenting as heart failure and diagnosed as recurrent myxoma by echocardiogram. Eur J Echocardiogr 2010; 11: E12. 6. Shanmugam G. Primary cardiac sarcoma. Eur J Cardiothorac Surg 2006; 29: 925–932. 7. Ekmektzoglou KA, Samelis GF and Xanthos T. Heart and tumors: location, metastasis, clinical manifestations, diagnostic approaches and therapeutic considerations. J Cardiovasc Med (Hagerstown) 2008; 9: 769–777. 8. Shapiro LM. Cardiac tumours: diagnosis and management [Review]. Heart 2001; 85: 218–222.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
