indian heart journal 67 (2015) 485–488
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Images in Cardiology
The evanescent right atrial mass Mohamad Lazkani *, Tomas Rivera-Bonilla, Moneer Eddin, Sudhakar Girotra, Ashish Pershad Department of Cardiology, Cavanagh Heart Center, Banner University Medical Center, Phoenix, AZ 85006, USA
article info
abstract
Article history:
An unusual cause of pulmonary emboli from an evanescent right atrial mass is described in
Received 9 April 2015
this case report. The systematic approach from initial presentation to a definite diagnosis of
Accepted 27 May 2015
a rare condition is described.
Available online 8 August 2015
# 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.
Keywords: Lymphoma Burkitt lymphoma Atrial mass Cardiac tumor
[(Fig._1)TD$IG] 1.
Case report
A 38-year-old Caucasian male with HIV on highly active antiretroviral therapy for 13 years and hepatitis C presented to the Emergency Department (ED) with a 1-week history of increasing dyspnea, tachycardia and palpitations without syncope or chest pain. Workup in the ED revealed an elevated D-Dimer-2.31 mg/ ml (normal 0.22–0.50 mcg/ml). This prompted a CTA which showed sub-segmental pulmonary emboli. A density in the right atrium and right ventricle was also noted [Fig. 1]. A transthoracic echocardiogram revealed a large mass measuring 6.9 cm 5.0 cm in the right atrium with a ‘‘cluster of grapes’’ appearance prolapsing into the RV in diastole through the tricuspid valve [Fig. 2]. A trans-esophageal echocardiogram [Fig. 3] and cardiac magnetic resonance imaging (CMR) were then performed to further characterize
Fig. 1 – Chest CT scan showing a right-atrial mass measuring 5.7 cm in diameter.
* Corresponding author at: Banner University Medical Center, Department of Cardiology, 1111 E McDowell Rd, Phoenix, Arizona 85006, USA. Tel.: +1 650 922 8439; fax: +1 602 839 5094. E-mail address: [email protected] (M. Lazkani). http://dx.doi.org/10.1016/j.ihj.2015.05.021 0019-4832/# 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.
[(Fig._2)TD$IG]
486
indian heart journal 67 (2015) 485–488
Fig. 2 – Transthoracic echocardiogram showing a large mass with a ‘‘grape cluster’’ appearance and filling the right atrium near completely.
and delineate the extent of the mass. CMR confirmed a lobulated mass filling the right atrium [Fig. 4a], which was isointense on double inversion recovery images [Fig. 4b] and hyperintense on triple inversion recovery images [Fig. 4c]. After administration of gadolinium based contrast, there was [(Fig._4)TD$IG]heterogeneous delayed enhancement [Fig. 4d].
[(Fig._3)TD$IG]
Fig. 3 – Transesophageal echocardiogram showing a large mass prolapsing through the tricuspid valve and filling the right atrium near completely.
Due to the concern about extracardiac extension of this mass on CT and MR, and the high likelihood that this might be a malignancy, the decision was made to obtain a tissue diagnosis to save this patient an open sternotomy.
Fig. 4 – (a) Steady state free precession cardiac magnetic resonance in a 4-chamber view showing a lobulated mass filling the right atrium. (b) Double inversion recovery cardiac magnetic resonance in a 4-chamber view showing an isointense right atrial mass. (c) Triple inversion recovery cardiac magnetic resonance in a 4-chamber view showing a hyperintense right atrial mass. (d) Post contrast delayed enhancement cardiac magnetic resonance in a 4-chamber view showing heterogeneous enhancement of the right atrial mass.
indian heart journal 67 (2015) 485–488
[(Fig._5)TD$IG]
[(Fig._6)TD$IG]
487
Fig. 6 – Transthoracic echocardiogram showing resolution of the right sided atrial mass after 6 weeks.
Fig. 5 – Hematoxilin and Eosin (H&E) stain (a) of cardiac mass at 40T magnification shows dense monomorphic lymphocytes consistent in appearance with a high-grade lymphoma. CD20 stain (b) at 40T magnification shows cell membranes stain brown indicating presence of CD20.
The cardiac mass was biopsied via trans-jugular approach and histopathologic examination revealed CD-20 positive Burkitt's lymphoma [Fig. 5]. Bone marrow biopsy demonstrated 80% normocellular bone marrow with progressive trilinear hematopoiesis and iron deficiency but no abnormal cells and only 5% lymphocytes. The patient subsequently underwent chemotherapy with Rituximab, Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (R-EPOCH). The patient's clinical symptoms of dyspnea, tachycardia and palpitations resolved completely at 6 weeks with disappearance of the mass [Fig. 6].
2.
Discussion
There have been a few cases described in the literature in which a lymphoma with cardiac involvement is the first
manifestation of HIV.1,2 HIV associated cardiac lymphomas when symptomatic typically present with heart failure in addition to the classically described-drenching night sweats and weight loss.3 With further characterization of cardiac masses, cardiac magnetic resonance imaging remains the gold standard. Although cardiac lymphoma can demonstrate a variety of imaging features on CMR, the combination of hyperintense signal on triple inversion recovery imaging and heterogeneous enhancement was important in suggesting tumor rather than thrombus. Tissue biopsy was performed to confirm a diagnosis, as management is impacted drastically. Furthermore, our patient was diagnosed with a sub-segmental pulmonary embolism. However, there have been reports in the literature where intracardiac Burkitt's lymphoma has mimicked acute pulmonary embolism.4 In addition to direct myocardial involvement, HIV-associated lymphoma can also be associated with pericardial disease. This can lead to symptoms masquerading as cardiac tamponade.5,6 This case demonstrates an unusual presentation of a rare disorder. Lymphoma associated with HIV with CD20 positivity has been shown to respond well to Rituximab in addition to Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone (CHOP) therapy.3 Cardiac involvement typically portends a poorer prognosis but that is in the context of heart failure and systemic involvement, unlike this case. If hemodynamic compromise occurs, surgical excision of the mass is often the mainstay of therapy.7
Conflicts of interest All authors have none to declare.
Acknowledgments We would like to acknowledge the following physicians for their contributions to this case report: David Paul1 MD, Robert Raschke2 MD, and Michael Morris3 MD. (1 Department of
488
indian heart journal 67 (2015) 485–488
Hematology and Oncology; 2 Department of Pulmonary and Critical Care Medicine; 3 Department of Radiology; Banner University Medical Center, Phoenix, Arizona 85006).
Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2015.05.021.
references
1. Iwahashi N, Nakatani S, Kakuchi H, Yamagishi M, Fukuchi K, Ishida Y. Cardiac tumor as an initial manifestation of acquired immunodeficiency syndrome. Circ J Official J Jpn Circulation Soc. 2005;69:243.
2. Duong M, Dubois C, Buisson M, et al. Non-hodgkin's lymphoma of the heart in patients infected with human immunodeficiency virus. Clin Cardiol. 1997;20:497–502. 3. Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large B-call lymphoma. N Engl J Med. 2002;346:235–242. 4. Fatimi S, Sheikh S, Shah Z, Shafiq M. Intracardiac burkitt's lymphoma mimicking acute pulmonary embolism. J Coll Physicians Surg Pak. 2006;16:536–537. 5. Kaul P, Javangula K. Burkitt lymphoma masquerading as cardiac tamponade. J Cardiothorac Surg. 2007;2:30. 6. Ikematsu W, Aso T, Hirota Y, et al. Long term remission of a case of Burkitt's lymphoma accompanied by cardiac tamponade treated by surgical resection and intensive chemotherapy. Rinsho Ketsueki. 1990;31:1731–1735. 7. Singh AS, Dave DJ, Thanvi S, Atre DA, Parikh P, Patel NH. Fatal secondary pulmonary hypertension due to cardiac involvement in AIDS-associated Burkitt's lymphoma. Indian J Med Sci. 2006;60:380.
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Images in Cardiology
The evanescent right atrial mass Mohamad Lazkani *, Tomas Rivera-Bonilla, Moneer Eddin, Sudhakar Girotra, Ashish Pershad Department of Cardiology, Cavanagh Heart Center, Banner University Medical Center, Phoenix, AZ 85006, USA
article info
abstract
Article history:
An unusual cause of pulmonary emboli from an evanescent right atrial mass is described in
Received 9 April 2015
this case report. The systematic approach from initial presentation to a definite diagnosis of
Accepted 27 May 2015
a rare condition is described.
Available online 8 August 2015
# 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.
Keywords: Lymphoma Burkitt lymphoma Atrial mass Cardiac tumor
[(Fig._1)TD$IG] 1.
Case report
A 38-year-old Caucasian male with HIV on highly active antiretroviral therapy for 13 years and hepatitis C presented to the Emergency Department (ED) with a 1-week history of increasing dyspnea, tachycardia and palpitations without syncope or chest pain. Workup in the ED revealed an elevated D-Dimer-2.31 mg/ ml (normal 0.22–0.50 mcg/ml). This prompted a CTA which showed sub-segmental pulmonary emboli. A density in the right atrium and right ventricle was also noted [Fig. 1]. A transthoracic echocardiogram revealed a large mass measuring 6.9 cm 5.0 cm in the right atrium with a ‘‘cluster of grapes’’ appearance prolapsing into the RV in diastole through the tricuspid valve [Fig. 2]. A trans-esophageal echocardiogram [Fig. 3] and cardiac magnetic resonance imaging (CMR) were then performed to further characterize
Fig. 1 – Chest CT scan showing a right-atrial mass measuring 5.7 cm in diameter.
* Corresponding author at: Banner University Medical Center, Department of Cardiology, 1111 E McDowell Rd, Phoenix, Arizona 85006, USA. Tel.: +1 650 922 8439; fax: +1 602 839 5094. E-mail address: [email protected] (M. Lazkani). http://dx.doi.org/10.1016/j.ihj.2015.05.021 0019-4832/# 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.
[(Fig._2)TD$IG]
486
indian heart journal 67 (2015) 485–488
Fig. 2 – Transthoracic echocardiogram showing a large mass with a ‘‘grape cluster’’ appearance and filling the right atrium near completely.
and delineate the extent of the mass. CMR confirmed a lobulated mass filling the right atrium [Fig. 4a], which was isointense on double inversion recovery images [Fig. 4b] and hyperintense on triple inversion recovery images [Fig. 4c]. After administration of gadolinium based contrast, there was [(Fig._4)TD$IG]heterogeneous delayed enhancement [Fig. 4d].
[(Fig._3)TD$IG]
Fig. 3 – Transesophageal echocardiogram showing a large mass prolapsing through the tricuspid valve and filling the right atrium near completely.
Due to the concern about extracardiac extension of this mass on CT and MR, and the high likelihood that this might be a malignancy, the decision was made to obtain a tissue diagnosis to save this patient an open sternotomy.
Fig. 4 – (a) Steady state free precession cardiac magnetic resonance in a 4-chamber view showing a lobulated mass filling the right atrium. (b) Double inversion recovery cardiac magnetic resonance in a 4-chamber view showing an isointense right atrial mass. (c) Triple inversion recovery cardiac magnetic resonance in a 4-chamber view showing a hyperintense right atrial mass. (d) Post contrast delayed enhancement cardiac magnetic resonance in a 4-chamber view showing heterogeneous enhancement of the right atrial mass.
indian heart journal 67 (2015) 485–488
[(Fig._5)TD$IG]
[(Fig._6)TD$IG]
487
Fig. 6 – Transthoracic echocardiogram showing resolution of the right sided atrial mass after 6 weeks.
Fig. 5 – Hematoxilin and Eosin (H&E) stain (a) of cardiac mass at 40T magnification shows dense monomorphic lymphocytes consistent in appearance with a high-grade lymphoma. CD20 stain (b) at 40T magnification shows cell membranes stain brown indicating presence of CD20.
The cardiac mass was biopsied via trans-jugular approach and histopathologic examination revealed CD-20 positive Burkitt's lymphoma [Fig. 5]. Bone marrow biopsy demonstrated 80% normocellular bone marrow with progressive trilinear hematopoiesis and iron deficiency but no abnormal cells and only 5% lymphocytes. The patient subsequently underwent chemotherapy with Rituximab, Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (R-EPOCH). The patient's clinical symptoms of dyspnea, tachycardia and palpitations resolved completely at 6 weeks with disappearance of the mass [Fig. 6].
2.
Discussion
There have been a few cases described in the literature in which a lymphoma with cardiac involvement is the first
manifestation of HIV.1,2 HIV associated cardiac lymphomas when symptomatic typically present with heart failure in addition to the classically described-drenching night sweats and weight loss.3 With further characterization of cardiac masses, cardiac magnetic resonance imaging remains the gold standard. Although cardiac lymphoma can demonstrate a variety of imaging features on CMR, the combination of hyperintense signal on triple inversion recovery imaging and heterogeneous enhancement was important in suggesting tumor rather than thrombus. Tissue biopsy was performed to confirm a diagnosis, as management is impacted drastically. Furthermore, our patient was diagnosed with a sub-segmental pulmonary embolism. However, there have been reports in the literature where intracardiac Burkitt's lymphoma has mimicked acute pulmonary embolism.4 In addition to direct myocardial involvement, HIV-associated lymphoma can also be associated with pericardial disease. This can lead to symptoms masquerading as cardiac tamponade.5,6 This case demonstrates an unusual presentation of a rare disorder. Lymphoma associated with HIV with CD20 positivity has been shown to respond well to Rituximab in addition to Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone (CHOP) therapy.3 Cardiac involvement typically portends a poorer prognosis but that is in the context of heart failure and systemic involvement, unlike this case. If hemodynamic compromise occurs, surgical excision of the mass is often the mainstay of therapy.7
Conflicts of interest All authors have none to declare.
Acknowledgments We would like to acknowledge the following physicians for their contributions to this case report: David Paul1 MD, Robert Raschke2 MD, and Michael Morris3 MD. (1 Department of
488
indian heart journal 67 (2015) 485–488
Hematology and Oncology; 2 Department of Pulmonary and Critical Care Medicine; 3 Department of Radiology; Banner University Medical Center, Phoenix, Arizona 85006).
Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2015.05.021.
references
1. Iwahashi N, Nakatani S, Kakuchi H, Yamagishi M, Fukuchi K, Ishida Y. Cardiac tumor as an initial manifestation of acquired immunodeficiency syndrome. Circ J Official J Jpn Circulation Soc. 2005;69:243.
2. Duong M, Dubois C, Buisson M, et al. Non-hodgkin's lymphoma of the heart in patients infected with human immunodeficiency virus. Clin Cardiol. 1997;20:497–502. 3. Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large B-call lymphoma. N Engl J Med. 2002;346:235–242. 4. Fatimi S, Sheikh S, Shah Z, Shafiq M. Intracardiac burkitt's lymphoma mimicking acute pulmonary embolism. J Coll Physicians Surg Pak. 2006;16:536–537. 5. Kaul P, Javangula K. Burkitt lymphoma masquerading as cardiac tamponade. J Cardiothorac Surg. 2007;2:30. 6. Ikematsu W, Aso T, Hirota Y, et al. Long term remission of a case of Burkitt's lymphoma accompanied by cardiac tamponade treated by surgical resection and intensive chemotherapy. Rinsho Ketsueki. 1990;31:1731–1735. 7. Singh AS, Dave DJ, Thanvi S, Atre DA, Parikh P, Patel NH. Fatal secondary pulmonary hypertension due to cardiac involvement in AIDS-associated Burkitt's lymphoma. Indian J Med Sci. 2006;60:380.
