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Primary cardiac lymphoma
1000
CASEREPORT MARGOLINET AL PRIMARYCARDIACLYMPHOMA
3. Stansel HC Jr. A new operation for d-loop transposition of the great vessels. Ann Thorac Surg 1975;19:565-7. 4. Pridjian AK, Mendelsohn A, Lupinetti FM, Beekman RH, Bove EL. The bidirectional Glenn shunt: staged reconstruction for the functional single ventricle. Ann Thorac Surg 1993;71:959-62. 5. Mayer JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castefiada AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992;103:444-51.
Primary Cardiac Lymphoma David A. Margolin, MD, Vladimir Fabian, MD, Uri Mintz, MD, and M a r k J. Botham, MD Departments of Surgery and Internal Medicine, Case Western Reserve University School of Medicine and Mt. Sinai Medical Center, Cleveland, Ohio A case of primary cardiac non-Hodgkin's B cell lymphoma is described in a patient presenting with obstructive right heart failure. Unlike the majority of cases, in this case the tumor was diagnosed ante mortem. The patient's history combined with the aggressive use of noninvasive echocardiography are helpful in diagnosing this rare lesion. (Ann Thorac Surg 1996;61:1000-1)
p
rimary cardiac l y m p h o m a , an extranodal m a l i g n a n t l y m p h o m a of any cell type involving only the heart a n d p e r i c a r d i u m without dissemination, is an extremely rare lesion, with fewer than 20 cases r e p o r t e d in the world literature [1-4]. The majority of these cases have b e e n d i a g n o s e d post mortem, with an increasing frequency seen in i m m u n o c o m p r o m i s e d individuals since 1987 [5-7]. W e r e p o r t a n o n i m m u n o s u p p r e s s e d patient with p r i m a r y cardiac l y m p h o m a who p r e s e n t e d with acute obstructive right heart failure.
Ann Thorac Surg 1996;61:1000-1
15.0 m m Hg. An inferior vena cavogram confirmed the presence of a mass p r o l a p s i n g into the inferior vena cava with extension inferiorly below the level of the hepatic veins (Fig 1). Cardiac catheterization was performed, revealing n o r m a l coronary a n a t o m y with the exception of collateral vessels that were perfusing the t u m o r from the right coronary artery. I m m e d i a t e l y after catheterization, significant a b d o m i nal pain developed, and the patient was found by radiographic evaluation to have i n t r a a b d o m i n a l free air. Emergently the patient was taken to the operating room, w h e r e he was f o u n d to have an anterior d u o d e n a l perforation. This was r e p a i r e d by G r a h a m plication. The patient b e c a m e p r o f o u n d l y hypotensive during the procedure, with a rapidly falling systolic b l o o d pressure that was u n r e s p o n s i v e to both volume and inotropic support. At sternotomy, the heart a p p e a r e d extremely cyanotic with m a r k e d l y d i m i n i s h e d contractility. The right atrium was massively dilated with extensive t u m o r involvement. The patient was placed on b y p a s s using a r i g h t - a n g l e d venous cannula placed in the superior vena cava and a second venous cannula t h r e a d e d to the retrohepatic inferior vena cava from the right femoral vein. U n d e r 28 m i n u t e s of h y p o t h e r m i c circulatory arrest the right atrium was o p e n e d and a t u m o r m e a s u r i n g 7 × 6 × 4.5 cm was f o u n d to originate from the right atrial free wall with extension through the tricuspid valve a n d into the inferior vena cava. The t u m o r with the attached right atrial wall a n d involved inferior vena cava was excised a n d reconstructed with a bovine pericardial patch to achieve a d e q u a t e surgical margins. Histologic evaluation of the mass revealed a nonH o d g k i n ' s B cell l y m p h o m a . I m m u n o p e r o x i d a s e staining d e m o n s t r a t e d positive t u m o r staining for c o m m o n leukocyte antigen a n d also L26 antigen, which is specific for B cell l y m p h o m a . The n e o p l a s m c o m p r i s e d dense sheets of large cells with p r o m i n e n t nucleoli a n d mitotic figures
The patient, a 63-year-old m a n with a prior history of m a l i g n a n t melanoma, clinically free of recurrence since 1980, p r e s e n t e d for evaluation of malaise, weight gain, and exertional dyspnea. Physical examination revealed m a r k e d right-sided heart failure with neck vein distention, ascites, and p e d a l edema. Liver function tests h a d e l e v a t e d results, with a s e r u m glutamic-oxaloacetic t r a n s a m i n a s e level of 109 lUlL, a s e r u m glutamic-pyruvic t r a n s a m i n a s e level of 113 IU/L, a n d a p r o t h r o m b i n time of 15.7 seconds. Transesophageal echocardiography demonstrated a 7.5-cm lobulated mass filling the right atrium. The mass p r o l a p s e d through the tricuspid valve, compressing the valve leaflets and p r o d u c i n g an area of high-velocity flow into the right ventricle with a m e a n gradient of Accepted for publication Oct 4, 1995. Address reprint requests to Dr Botham, Department of Surgery, Mt. Sinai Medical Center, One Mt. Sinai Dr, Cleveland, OH 44106. © 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Fig 1. Right atrial involvement by the tumor. 0003-4975/96/$15.00 SSDI 0003-4975(95)00981-7
Ann Thorac Surg 1996;61:1001-3
that insinuated into the u n d e r l y i n g myocardial fibers. The t u m o r stained negative for CD3 (T cells) a n d MAK 6, which is an epithelial cell marker. The margins of resection were free of tumor. The patient initially d i d well postoperatively. O n postoperative day 9 he u n d e r w e n t a b a r i u m swallow, which s h o w e d no evidence of a d u o d e n a l leak, a n d he was started on oral alimentation. O n postoperative day 20 an acute a b d o m e n developed, a n d the patient was found at l a p a r o t o m y to have a recurrent d u o d e n a l perforation. Despite operative repair of the perforation a n d b r o a d s p e c t r u m antibiotic coverage the patient b e c a m e septic; multisystem organ failure developed, a n d he d i e d on postoperative day 30.
CASE REPORT SOMMERSET AL RESECTION OF A PRIMARYCARDIACLYMPHOMA
4. 5. 6.
7. 8.
1001
toms of B-cell malignant non-Hodgkin lymphoma involving the heart. Haematologica 1993;78:332-4. Roller MB, Manoharan A, Lvoff R. Primary cardiac lymphoma. Acta Haematol 1991;85:47-8. Constantino A, West TE, Gupta M, Loghmanee F. Primary cardiac lymphoma in a patient with acquired immune deftciency syndrome. Cancer 1987;60:2801-5. Gaurner J, Brynes RK, Chan WC, Birdsong G, Hertzler G. Primary non-Hodgkin's lymphoma of the heart in two patients with acquired immunodeficiency syndrome. Arch Pathol Lab Med 1987;111:254-6. Rodenburg CJ, Kluin P, Maes A, Paul LC. Malignant lymphoma confined to the heart, 13 years after a cadaver kidney transplant [Letter]. N Engl J Med 1985;313:122. McDonnell PJ, Mann RB, Buckley BH. Involvement of the heart by malignant lymphoma: a clinicopathologic study. Cancer 1982;49:944-51.
Comment Cardiac involvement in m a l i g n a n t l y m p h o m a s is relatively common, with 20% of patients showing cardiac involvement at the time of death [8]. Primary cardiac l y m p h o m a s in n o n i m m u n o c o m p r o m i s e d individuals are exceedingly rare. Before 1987 there were only 20 r e p o r t e d cases of p r i m a r y cardiac l y m p h o m a s in the Englishlanguage literature. Since 1987 there has b e e n a significant increase in the presence of this lesion associated with i m m u n o s u p p r e s s i o n , both in individuals iatrogenically i m m u n o s u p p r e s s e d for solid organ transplantation and in patients positive for h u m a n immunodeficiency virus or patients with acquired immunodeficiency synd r o m e [4-7]. A l t h o u g h all histologic types of l y m p h o m a involve the heart, those of the diffuse large cell or immunoblastic B cell subtype p r e d o m i n a t e . Burkitt's l y m p h o m a of the heart has b e e n r e p o r t e d on only two occasions. Most patients with a p r i m a r y cardiac l y m p h o m a have a clinical course acute in onset with a r a p i d demise. Early diagnosis m a y best be m a d e by echocardiography. Transesophageal e c h o c a r d i o g r a p h y allows for architectural delineation of (1) single or multiple c h a m b e r involvement, (2) the focal or diffuse nature of the disease, a n d (3) the n e e d for or feasibility of surgical resection. C o m b i n e d c h e m o t h e r a p y a n d radiation t h e r a p y m a y be a p p r o p r i a t e in early stages. Obstructive s y m p t o m s or congestive heart failure often suggest mechanical inflow/ outflow compromise. Surgical exploration is m a r k e d l y a i d e d by h y p o t h e r m i c circulatory arrest, which allows for a bloodless field to examine the extent of t u m o r involvem e n t in the vena cava. Resection a n d reconstruction, if possible, m a y then be undertaken, assuring a tumor-free margin.
References 1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Arias of tumor pathology, 2nd series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978: 99 -100. 2. Chou S, Arkles LB, Gill GD, et al. Primary lymphoma of the heart. A case report. Cancer 1983;52:744-7. 3. Bolis S, Bregani ER, Rossini F, Schiavina, Pogliani EM. Atrial flutter followed by sick sinus syndrome as presenting symp© 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Primary Cardiac Lymphoma: Echocardiographic Characterization and Successful Resection K. Eric Sommers, MD, Daniel Edmundowicz, MD, William E. Katz, MD, a n d Brack G. Hattler, MD, PhD Divisions of Cardiothoracic Surgery and Cardiology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
We present a case of primary cardiac lymphoma in a patient with dyspnea and hypoxemia. Transesophageal echocardiography revealed a large right atrial mass and an atrial septal aneurysm with right-to-left shunting through a patent foramen ovale. The patient underwent resection and atrial reconstruction. Pathology was a B cell lymphoma with diffuse large cell histology. There was no evidence of extracardiac involvement, and the patient is well 3 months postoperatively with a normal transthoracic echocardiogram.
(Ann Thorac Surg 1996;61:1001-3)
p
rimary cardiac tumors, defined as t u m o r s involving only the heart or pericardium, are rare malignancies with a r e p o r t e d incidence 0.0017% to 0.28% [1, 2]. Of p r i m a r y cardiac tumors, p r i m a r y cardiac l y m p h o m a is extremely rare, with an incidence of 1.3% [1]. Primary cardiac l y m p h o m a m a y p r e s e n t as congestive heart failure, pericardial effusion, arrhythmias, or superior vena cava s y n d r o m e [3, 4]. The majority of patients die within weeks after diagnosis or are d i a g n o s e d at autopsy. This case of p r i m a r y cardiac l y m p h o m a is u n u s u a l in that it was h e r a l d e d b y an acute presentation of d y s p n e a a n d h y p o x e m i a from shunting through a p a t e n t foramen ovale. Accepted for publication Sep 13, 1995. Address reprint requests to Dr Katz, Divisionof Cardiology,Universityof Pittsburgh Medical Center, $563 Scaife Hall, 200 Lothrop St, Pittsburgh, PA 15213-2582. 0003-4975/96/$15.00 SSDI 0003-4975(95)01033-5
CASEREPORT MARGOLINET AL PRIMARYCARDIACLYMPHOMA
3. Stansel HC Jr. A new operation for d-loop transposition of the great vessels. Ann Thorac Surg 1975;19:565-7. 4. Pridjian AK, Mendelsohn A, Lupinetti FM, Beekman RH, Bove EL. The bidirectional Glenn shunt: staged reconstruction for the functional single ventricle. Ann Thorac Surg 1993;71:959-62. 5. Mayer JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castefiada AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992;103:444-51.
Primary Cardiac Lymphoma David A. Margolin, MD, Vladimir Fabian, MD, Uri Mintz, MD, and M a r k J. Botham, MD Departments of Surgery and Internal Medicine, Case Western Reserve University School of Medicine and Mt. Sinai Medical Center, Cleveland, Ohio A case of primary cardiac non-Hodgkin's B cell lymphoma is described in a patient presenting with obstructive right heart failure. Unlike the majority of cases, in this case the tumor was diagnosed ante mortem. The patient's history combined with the aggressive use of noninvasive echocardiography are helpful in diagnosing this rare lesion. (Ann Thorac Surg 1996;61:1000-1)
p
rimary cardiac l y m p h o m a , an extranodal m a l i g n a n t l y m p h o m a of any cell type involving only the heart a n d p e r i c a r d i u m without dissemination, is an extremely rare lesion, with fewer than 20 cases r e p o r t e d in the world literature [1-4]. The majority of these cases have b e e n d i a g n o s e d post mortem, with an increasing frequency seen in i m m u n o c o m p r o m i s e d individuals since 1987 [5-7]. W e r e p o r t a n o n i m m u n o s u p p r e s s e d patient with p r i m a r y cardiac l y m p h o m a who p r e s e n t e d with acute obstructive right heart failure.
Ann Thorac Surg 1996;61:1000-1
15.0 m m Hg. An inferior vena cavogram confirmed the presence of a mass p r o l a p s i n g into the inferior vena cava with extension inferiorly below the level of the hepatic veins (Fig 1). Cardiac catheterization was performed, revealing n o r m a l coronary a n a t o m y with the exception of collateral vessels that were perfusing the t u m o r from the right coronary artery. I m m e d i a t e l y after catheterization, significant a b d o m i nal pain developed, and the patient was found by radiographic evaluation to have i n t r a a b d o m i n a l free air. Emergently the patient was taken to the operating room, w h e r e he was f o u n d to have an anterior d u o d e n a l perforation. This was r e p a i r e d by G r a h a m plication. The patient b e c a m e p r o f o u n d l y hypotensive during the procedure, with a rapidly falling systolic b l o o d pressure that was u n r e s p o n s i v e to both volume and inotropic support. At sternotomy, the heart a p p e a r e d extremely cyanotic with m a r k e d l y d i m i n i s h e d contractility. The right atrium was massively dilated with extensive t u m o r involvement. The patient was placed on b y p a s s using a r i g h t - a n g l e d venous cannula placed in the superior vena cava and a second venous cannula t h r e a d e d to the retrohepatic inferior vena cava from the right femoral vein. U n d e r 28 m i n u t e s of h y p o t h e r m i c circulatory arrest the right atrium was o p e n e d and a t u m o r m e a s u r i n g 7 × 6 × 4.5 cm was f o u n d to originate from the right atrial free wall with extension through the tricuspid valve a n d into the inferior vena cava. The t u m o r with the attached right atrial wall a n d involved inferior vena cava was excised a n d reconstructed with a bovine pericardial patch to achieve a d e q u a t e surgical margins. Histologic evaluation of the mass revealed a nonH o d g k i n ' s B cell l y m p h o m a . I m m u n o p e r o x i d a s e staining d e m o n s t r a t e d positive t u m o r staining for c o m m o n leukocyte antigen a n d also L26 antigen, which is specific for B cell l y m p h o m a . The n e o p l a s m c o m p r i s e d dense sheets of large cells with p r o m i n e n t nucleoli a n d mitotic figures
The patient, a 63-year-old m a n with a prior history of m a l i g n a n t melanoma, clinically free of recurrence since 1980, p r e s e n t e d for evaluation of malaise, weight gain, and exertional dyspnea. Physical examination revealed m a r k e d right-sided heart failure with neck vein distention, ascites, and p e d a l edema. Liver function tests h a d e l e v a t e d results, with a s e r u m glutamic-oxaloacetic t r a n s a m i n a s e level of 109 lUlL, a s e r u m glutamic-pyruvic t r a n s a m i n a s e level of 113 IU/L, a n d a p r o t h r o m b i n time of 15.7 seconds. Transesophageal echocardiography demonstrated a 7.5-cm lobulated mass filling the right atrium. The mass p r o l a p s e d through the tricuspid valve, compressing the valve leaflets and p r o d u c i n g an area of high-velocity flow into the right ventricle with a m e a n gradient of Accepted for publication Oct 4, 1995. Address reprint requests to Dr Botham, Department of Surgery, Mt. Sinai Medical Center, One Mt. Sinai Dr, Cleveland, OH 44106. © 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Fig 1. Right atrial involvement by the tumor. 0003-4975/96/$15.00 SSDI 0003-4975(95)00981-7
Ann Thorac Surg 1996;61:1001-3
that insinuated into the u n d e r l y i n g myocardial fibers. The t u m o r stained negative for CD3 (T cells) a n d MAK 6, which is an epithelial cell marker. The margins of resection were free of tumor. The patient initially d i d well postoperatively. O n postoperative day 9 he u n d e r w e n t a b a r i u m swallow, which s h o w e d no evidence of a d u o d e n a l leak, a n d he was started on oral alimentation. O n postoperative day 20 an acute a b d o m e n developed, a n d the patient was found at l a p a r o t o m y to have a recurrent d u o d e n a l perforation. Despite operative repair of the perforation a n d b r o a d s p e c t r u m antibiotic coverage the patient b e c a m e septic; multisystem organ failure developed, a n d he d i e d on postoperative day 30.
CASE REPORT SOMMERSET AL RESECTION OF A PRIMARYCARDIACLYMPHOMA
4. 5. 6.
7. 8.
1001
toms of B-cell malignant non-Hodgkin lymphoma involving the heart. Haematologica 1993;78:332-4. Roller MB, Manoharan A, Lvoff R. Primary cardiac lymphoma. Acta Haematol 1991;85:47-8. Constantino A, West TE, Gupta M, Loghmanee F. Primary cardiac lymphoma in a patient with acquired immune deftciency syndrome. Cancer 1987;60:2801-5. Gaurner J, Brynes RK, Chan WC, Birdsong G, Hertzler G. Primary non-Hodgkin's lymphoma of the heart in two patients with acquired immunodeficiency syndrome. Arch Pathol Lab Med 1987;111:254-6. Rodenburg CJ, Kluin P, Maes A, Paul LC. Malignant lymphoma confined to the heart, 13 years after a cadaver kidney transplant [Letter]. N Engl J Med 1985;313:122. McDonnell PJ, Mann RB, Buckley BH. Involvement of the heart by malignant lymphoma: a clinicopathologic study. Cancer 1982;49:944-51.
Comment Cardiac involvement in m a l i g n a n t l y m p h o m a s is relatively common, with 20% of patients showing cardiac involvement at the time of death [8]. Primary cardiac l y m p h o m a s in n o n i m m u n o c o m p r o m i s e d individuals are exceedingly rare. Before 1987 there were only 20 r e p o r t e d cases of p r i m a r y cardiac l y m p h o m a s in the Englishlanguage literature. Since 1987 there has b e e n a significant increase in the presence of this lesion associated with i m m u n o s u p p r e s s i o n , both in individuals iatrogenically i m m u n o s u p p r e s s e d for solid organ transplantation and in patients positive for h u m a n immunodeficiency virus or patients with acquired immunodeficiency synd r o m e [4-7]. A l t h o u g h all histologic types of l y m p h o m a involve the heart, those of the diffuse large cell or immunoblastic B cell subtype p r e d o m i n a t e . Burkitt's l y m p h o m a of the heart has b e e n r e p o r t e d on only two occasions. Most patients with a p r i m a r y cardiac l y m p h o m a have a clinical course acute in onset with a r a p i d demise. Early diagnosis m a y best be m a d e by echocardiography. Transesophageal e c h o c a r d i o g r a p h y allows for architectural delineation of (1) single or multiple c h a m b e r involvement, (2) the focal or diffuse nature of the disease, a n d (3) the n e e d for or feasibility of surgical resection. C o m b i n e d c h e m o t h e r a p y a n d radiation t h e r a p y m a y be a p p r o p r i a t e in early stages. Obstructive s y m p t o m s or congestive heart failure often suggest mechanical inflow/ outflow compromise. Surgical exploration is m a r k e d l y a i d e d by h y p o t h e r m i c circulatory arrest, which allows for a bloodless field to examine the extent of t u m o r involvem e n t in the vena cava. Resection a n d reconstruction, if possible, m a y then be undertaken, assuring a tumor-free margin.
References 1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Arias of tumor pathology, 2nd series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978: 99 -100. 2. Chou S, Arkles LB, Gill GD, et al. Primary lymphoma of the heart. A case report. Cancer 1983;52:744-7. 3. Bolis S, Bregani ER, Rossini F, Schiavina, Pogliani EM. Atrial flutter followed by sick sinus syndrome as presenting symp© 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Primary Cardiac Lymphoma: Echocardiographic Characterization and Successful Resection K. Eric Sommers, MD, Daniel Edmundowicz, MD, William E. Katz, MD, a n d Brack G. Hattler, MD, PhD Divisions of Cardiothoracic Surgery and Cardiology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
We present a case of primary cardiac lymphoma in a patient with dyspnea and hypoxemia. Transesophageal echocardiography revealed a large right atrial mass and an atrial septal aneurysm with right-to-left shunting through a patent foramen ovale. The patient underwent resection and atrial reconstruction. Pathology was a B cell lymphoma with diffuse large cell histology. There was no evidence of extracardiac involvement, and the patient is well 3 months postoperatively with a normal transthoracic echocardiogram.
(Ann Thorac Surg 1996;61:1001-3)
p
rimary cardiac tumors, defined as t u m o r s involving only the heart or pericardium, are rare malignancies with a r e p o r t e d incidence 0.0017% to 0.28% [1, 2]. Of p r i m a r y cardiac tumors, p r i m a r y cardiac l y m p h o m a is extremely rare, with an incidence of 1.3% [1]. Primary cardiac l y m p h o m a m a y p r e s e n t as congestive heart failure, pericardial effusion, arrhythmias, or superior vena cava s y n d r o m e [3, 4]. The majority of patients die within weeks after diagnosis or are d i a g n o s e d at autopsy. This case of p r i m a r y cardiac l y m p h o m a is u n u s u a l in that it was h e r a l d e d b y an acute presentation of d y s p n e a a n d h y p o x e m i a from shunting through a p a t e n t foramen ovale. Accepted for publication Sep 13, 1995. Address reprint requests to Dr Katz, Divisionof Cardiology,Universityof Pittsburgh Medical Center, $563 Scaife Hall, 200 Lothrop St, Pittsburgh, PA 15213-2582. 0003-4975/96/$15.00 SSDI 0003-4975(95)01033-5