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Primary cardiac precursor B lymphoblastic lymphoma in a child
Cardiovascular Pathology 13 (2004) 116 – 119
Primary cardiac precursor B lymphoblastic lymphoma in a child A case report and review of the literature Deepa Bassia, Benjamin J. Lentznerb, Ralph S. Moscac, Bachir Alobeid a,* a
Department of Pathology, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, 14-229 Vanderbilt Clinic, New York, NY 10032, USA b Department of Pediatric Cardiology, College of Physicians and Surgeons, Columbia University, New York, NY, USA c Department of Pediatric Cardiac Surgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA Received 3 June 2003; received in revised form 29 October 2003; accepted 3 November 2003
Abstract We describe an extremely rare case of primary cardiac precursor B lymphoblastic lymphoma (B-LBL) in a 10-year-old boy who presented with nonspecific complaints of fatigue and vomiting for 2 weeks and an episode of syncope. Chest X-ray showed cardiomegaly and echocardiography revealed a large right atrial mass, which was successfully resected. Pathology showed precursor B-LBL arising in the heart and there was no evidence of disease at any extracardiac site. A brief review of the literature is also presented. D 2004 Elsevier Inc. All rights reserved. Keywords: Primary cardiac lymphoma; Lymphoblastic lymphoma; Cardiac tumor; Cardiac neoplasm
1. Introduction Primary cardiac lymphoma (PCL) is defined as an extranodal non-Hodgkin’s lymphoma of any cell type, exclusively located in the heart and/or pericardium with no evidence of extracardiac dissemination [1,2]. It is very rare in immunocompetent patients and is reported more frequently in immunocompromised patients [2]. A majority of the cases reported in the English literature are described in adults and elderly patients and are diffuse large B cell lymphomas (DLBCL). Only very rare cases are reported in children. Lymphoblastic lymphoma constitutes approximately one third to one half of all pediatric non-Hodgkin’s lymphomas, 90% of which are of precursor T cell lineage and only 10% are of precursor B cell lineage. Precursor B lymphoblastic lymphoma (B-LBL) most commonly involves the skin, bone, soft tissue, and lymph nodes, whereas mediastinal masses are uncommon. Leukemic presentation with involvement of the peripheral blood and bone marrow is most common [3]. In this report, we describe an extremely rare case of primary cardiac precursor B-LBL arising in a 10-year-old * Corresponding author. Fax: +1-212-305-2301. E-mail address: [email protected] (J. Lentzner). 1054-8807/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(03)00131-5
boy, probably the first well-documented case in a 10-yearold boy in the English literature. A brief review of the literature is also presented.
2. Report of a case The patient is a 10-year-old boy who had an unremarkable past medical history. He presented with nonspecific complaints of fatigue and vomiting for 2 weeks and had one episode of syncope prior to hospital admission. Chest X-ray showed cardiomegaly, and a transthoracic echocardiogram revealed a large heterogeneous right atrial mass protruding through the tricuspid valve into the right ventricle (Fig. 1). During surgery, a solitary and pedunculated intracavitary mass was found in the right atrium, attached via a stalk to the medial aspect of right atrium and superior vena cava junction (Fig. 2). Using femoral venous and superior vena caval cannulation, the mass was successfully resected with adjacent portions of right atrium and superior vena cava. Good macroscopic margins were obtained. The remaining defect was reconstructed with autologous atrial tissue. No other masses or enlarged lymph nodes were present. After surgical resection and final pathologic diagnosis of B-LBL, patient was treated
D. Bassi et al. / Cardiovascular Pathology 13 (2004) 116–119
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Fig. 1. Apical four chamber view of the heart via echocardiography. Note the large heterogeneous mass in the right atrium (RA) protruding through the tricuspid valve into the right ventricle (RV). LA (left atrium); LV (Left ventricle).
with chemotherapy. He is well and in complete remission a year after initial diagnosis.
Fig. 3. The resected mass was multilobulated and hemorrhagic.
CD10 (Novocastra, Newcastle upon Tyn, UK), and TdT (Supertechs, Bethesda, MD) were applied. 3. Materials and methods Fresh tissue was used for flow cytometric analysis and analyzed on an FACS Calibur instrument (BD Biosciences). Portions of tissue were fixed in formalin and embedded in paraffin, and H&E stained sections were used for routine histomorphology. Additional sections of paraffin embedded tissue were used for immunohistochemical staining, which was performed on an automated immunostainer (Dako, Carpinteria, CA) using an avidin– biotin complex technique. Antibodies against the antigens CD20, CD79a, CD3 (Dako),
Fig. 2. Intrathoracic view of the right atrium. The tumor (arrow, tan mass) is seen superiorly, and the thin wall of the distended right atrial appendage reveals a hemorrhagic mass.
4. Pathologic findings The lesion was a multilobulated mass of smooth tissue measuring 6.5 cm in maximum dimension with a hemorrhagic cut surface (Fig. 3). Histology showed a diffuse proliferation of medium-sized immature lymphoid cells with a high nuclear/cytoplasmic ratio, an immature chromatin pattern, and inconspicuous nucleoli (Fig. 4). Brisk mitotic activity was seen with focal starry sky appearance. The immature lymphoid cells infiltrated the myocardial tissue and stained diffusely with the antigens CD79a, CD10, and
Fig. 4. Diffuse proliferation of lymphoblastic cells (H&E, 60).
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D. Bassi et al. / Cardiovascular Pathology 13 (2004) 116–119
Fig. 5. Lymphoblasts are diffusely staining with anti-CD79a (immunoperoxidase, 40).
Fig. 7. Numerous lymphoblasts are positive for the nuclear antigen TdT (immunoperoxidase, 40).
TdT (Figs. 5– 7) and were negative for the CD20 antigen. Similarly, flow cytometric analysis revealed a CD45 dim population of immature lymphoid cells positive for the antigens CD19, cytoplasmic CD22 and CD79a, CD10, and nuclear TdT. The cells were negative for the CD20 antigen and did not express surface light chain immunoglobulins. The morphology and immunophenotype of the neoplastic cells were diagnostic of precursor B-LBL. Examination of the bone marrow and peripheral blood showed no evidence of precursor B lymphoblastic leukemia/lymphoma.
and hamartomas [4,5]. Malignant primary cardiac tumors are a minority, most of which represent various sarcomas. PCL is very rare in immunocompetent patients, comprising approximately 2% of all primary cardiac neoplasms and less than 0.5% of all extranodal lymphomas [2,4]. On the other hand, secondary involvement of the heart by malignant non-Hodgkin’s lymphoma is not uncommon and can be found in 15 – 30% of autopsy series [1]. PCL has been encountered with increasing frequency in patients with AIDS or other severe immunodeficiency states. The vast majority of cases are reported in adult and elderly patients, with slight predominance in males [2]. Chest pain and dyspnea are the most common presenting symptoms. Most lesions are solitary intracavitary tumoral masses involving the right atrium, majority of which represent DLBCL [2]. PCL in children is exceedingly rare. Our review of the English literature revealed few case reports of cardiac lymphoma in children, arising or presenting primarily in the heart (Table 1). However, the exact type and lineage of lymphoma and the extent of disease at presentation were not well documented in all cases. To the best of our knowledge, three cases of ‘‘cardiac lymphoblastic lymphoma’’ were previously reported in the English literature. One case was reported in a 13-year-old girl and was apparently of precursor B cell type [6]. Similar to our case, the pedunculated tumor mass arose from the right atrial wall, occupied the right atrium, and protruded into the right ventricle. However, this case did not fulfill the generally accepted strict definition of PCL since at presentation there was clinical evidence of disease in multiple extracardiac sites. The second case of ‘‘non-Hodgkin’s lymphoma of lymphoblastic type’’ involving the heart was reported in an 11-year-old boy [7]. In this case, the exact phenotype of the lymphoma (precursor B vs. T) was not documented. The hard and nodular tumor mass infiltrated the right atrial wall, extending through the pericardium to the right hilum. A third case of cardiac B-LBL was reported in an abstract form [11] in a 12-year-old boy. Here,
5. Discussion Primary cardiac tumors are rare and most are benign neoplasms. Myxomas are the most common and the rest include fibromas, lipomatous tumors, fibroelastic papillomas,
Fig. 6. Lymphoblasts are diffusely staining with anti-CD10 (immunoperoxidase, 40).
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Table 1 Summary of clinical and pathologic characteristics of cases of pediatric PCL reported in the literature and the current case Case no.
Age (years)/sex
Lymphoma type
Location
Outcome
Reference
1 2
13/female 13/male
Heart, right cheek, palate, and distal tibia ‘‘Left atrium’’
Relapsed Complete remission
[6] [8]
3 4
9/male 11/male
B-LBL ‘‘Small, noncleaved cell lymphoma’’ Burkitt lymphoma ‘‘Lymphoblastic lymphoma’’
Heart Right atrial mass extending through the pericardium into the hilum ‘‘Tumor infiltrating the right atrium, right ventricle, and lateral side of the left ventricle’’ Right and left atria, right suprahilar lymph nodes, pericardial and pleural effusions, and two areas of soft tissue in left chest wall Mass around the diaphragm involving the right heart border
Complete remission Dead
[2] [7]
Dead
[9]
In remission
[11]
In remission
[10]
Right intracavitary mass
Complete remission
Index case
5
8/?
‘‘Malignant non-Hodgkin’s lymphoma, type B’’
6
12/male
B cell lymphoblastic lymphoma
7
22 mo/female
8
10/male
B cell polymorphic post transplant lymphoma and recurrence (EBV positive ) B-LBL
three mass lesions were described: two in the right atrium and one in the left atrium. In addition, however, prominent right suprahilar lymph node enlargement, two nodules in the soft tissues of the left chest wall, and pericardial and pleural effusions were all described at presentation. Again, this case did not fulfill the strict definition of PCL. Other cases of PCL reported in children included a case of primary cardiac Burkitt’s lymphoma in a 9-year-old boy [2], a case of ‘‘small, noncleaved cell lymphoma’’ in a 13year-old boy [8], and a case of ‘‘highly malignant nonHodgkin’s lymphoma’’ in an 8-year-old child [9]. A case of polymorphic B cell posttransplant lymphoproliferative disorder developing around the diaphragm and secondarily involving the heart was also reported [10]. In our case, the intracavitary tumor mass was confined to the heart and showed immunomorphology diagnostic of a precursor B-LBL. There was no evidence of disease at any extracardiac site. Therefore, this is probably the first welldocumented case in a child. Histogenesis of PCL in immunocompetent patients is still unknown. There are different possible explanations for how PCL develop. Lymphomas can arise virtually in any extranodal site. In certain extranodal tissues, lymphomas arise from acquired extranodal lymphoid tissue after chronic inflammation and through a transformation process. However, association with a chronic inflammatory process and development of an acquired extranodal lymphoid tissue has not been well demonstrated in PCL. Alternatively, PCL may represent a metastasis or seeding from an occult and clinically undetectable lymphoma in a remote location from the heart. Further studies are clearly needed to better understand the histogenesis of PCL. In conclusion, PCLs are exceedingly rare in children and difficult to diagnose due to nonspecific clinical manifesta-
tions. This report shows that PCL should be considered in the differential diagnosis and work up of a child with cardiac neoplasm and that precursor B-LBL, a potentially curable disease, can present in a very unusual manner and site.
References [1] Burke A, Virmani R. Atlas of tumor pathology: tumors of the heart and great vessels. Third series. Fascicle 16. Washington, DC: Armed forces Institute of Pathology, 1996. pp. 1 – 11, 171 – 7. [2] Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier J-F, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of literature. Virchows Arch 2002;441:456 – 61. [3] Jaffe ES, Harris NL, Stein H, Vardiman JW. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Precursor B lymphoblastic leukemia/ lymphoblastic lymphoma. Tumors of hematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2001. pp. 109 – 11. [4] Fernandes F, Soufen HN, Ianni BM, Arteaga E, Ramires FJ, Mady C. Primary neoplasms of the heart. Clinical and histological presentation of 50 cases. Arq Bras Cardiol 2001;76(3):231 – 7. [5] Tazelaar HD, Locke TJ, Mc Gregor CGA. Pathology of surgically excised primary cardiac tumors. Mayo Clinic Proc 1992;67:957 – 65. [6] Kuo TT, Yang CP, Lin CH, Chang CH. Lymphoblastic lymphoma presenting as a huge intracavitary cardiac tumor causing heart failure. Pediatr Pathol 1987;7:341 – 9. [7] Rafeeq B, Narayandas K, Aboobacker CM. Non Hodgkin’s lymphoma presenting as cardiac tumor. Indian Pediatr 2001;38:543 – 5. [8] Moore JA, De Ran BP, Minor R, Arthur J, Fraker TD. Transesophageal echocardiographic evaluation of intracardiac lymphoma. Am Heart J 1992;124:514 – 6. [9] Ztot S, Cherradi R, Haddour L, Belhaj S, Kettani F, Benmimoun EG, Arharbi M. Primary cardiac lymphoma. Report of a case. Arch Mal Coeur Vaiss 2002;95(1):61 – 4. [10] Agarwala B, Rubin CM. Intracardiac lymphoma in a child; successful treatment with chemotherapy alone. Pediatr Cardiol 2000;21:401 – 2. [11] Das P, Caucao M, Weitzman S, et al. J Pediatr Hematol Oncol 2003; 25(4):511.
Primary cardiac precursor B lymphoblastic lymphoma in a child A case report and review of the literature Deepa Bassia, Benjamin J. Lentznerb, Ralph S. Moscac, Bachir Alobeid a,* a
Department of Pathology, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, 14-229 Vanderbilt Clinic, New York, NY 10032, USA b Department of Pediatric Cardiology, College of Physicians and Surgeons, Columbia University, New York, NY, USA c Department of Pediatric Cardiac Surgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA Received 3 June 2003; received in revised form 29 October 2003; accepted 3 November 2003
Abstract We describe an extremely rare case of primary cardiac precursor B lymphoblastic lymphoma (B-LBL) in a 10-year-old boy who presented with nonspecific complaints of fatigue and vomiting for 2 weeks and an episode of syncope. Chest X-ray showed cardiomegaly and echocardiography revealed a large right atrial mass, which was successfully resected. Pathology showed precursor B-LBL arising in the heart and there was no evidence of disease at any extracardiac site. A brief review of the literature is also presented. D 2004 Elsevier Inc. All rights reserved. Keywords: Primary cardiac lymphoma; Lymphoblastic lymphoma; Cardiac tumor; Cardiac neoplasm
1. Introduction Primary cardiac lymphoma (PCL) is defined as an extranodal non-Hodgkin’s lymphoma of any cell type, exclusively located in the heart and/or pericardium with no evidence of extracardiac dissemination [1,2]. It is very rare in immunocompetent patients and is reported more frequently in immunocompromised patients [2]. A majority of the cases reported in the English literature are described in adults and elderly patients and are diffuse large B cell lymphomas (DLBCL). Only very rare cases are reported in children. Lymphoblastic lymphoma constitutes approximately one third to one half of all pediatric non-Hodgkin’s lymphomas, 90% of which are of precursor T cell lineage and only 10% are of precursor B cell lineage. Precursor B lymphoblastic lymphoma (B-LBL) most commonly involves the skin, bone, soft tissue, and lymph nodes, whereas mediastinal masses are uncommon. Leukemic presentation with involvement of the peripheral blood and bone marrow is most common [3]. In this report, we describe an extremely rare case of primary cardiac precursor B-LBL arising in a 10-year-old * Corresponding author. Fax: +1-212-305-2301. E-mail address: [email protected] (J. Lentzner). 1054-8807/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(03)00131-5
boy, probably the first well-documented case in a 10-yearold boy in the English literature. A brief review of the literature is also presented.
2. Report of a case The patient is a 10-year-old boy who had an unremarkable past medical history. He presented with nonspecific complaints of fatigue and vomiting for 2 weeks and had one episode of syncope prior to hospital admission. Chest X-ray showed cardiomegaly, and a transthoracic echocardiogram revealed a large heterogeneous right atrial mass protruding through the tricuspid valve into the right ventricle (Fig. 1). During surgery, a solitary and pedunculated intracavitary mass was found in the right atrium, attached via a stalk to the medial aspect of right atrium and superior vena cava junction (Fig. 2). Using femoral venous and superior vena caval cannulation, the mass was successfully resected with adjacent portions of right atrium and superior vena cava. Good macroscopic margins were obtained. The remaining defect was reconstructed with autologous atrial tissue. No other masses or enlarged lymph nodes were present. After surgical resection and final pathologic diagnosis of B-LBL, patient was treated
D. Bassi et al. / Cardiovascular Pathology 13 (2004) 116–119
117
Fig. 1. Apical four chamber view of the heart via echocardiography. Note the large heterogeneous mass in the right atrium (RA) protruding through the tricuspid valve into the right ventricle (RV). LA (left atrium); LV (Left ventricle).
with chemotherapy. He is well and in complete remission a year after initial diagnosis.
Fig. 3. The resected mass was multilobulated and hemorrhagic.
CD10 (Novocastra, Newcastle upon Tyn, UK), and TdT (Supertechs, Bethesda, MD) were applied. 3. Materials and methods Fresh tissue was used for flow cytometric analysis and analyzed on an FACS Calibur instrument (BD Biosciences). Portions of tissue were fixed in formalin and embedded in paraffin, and H&E stained sections were used for routine histomorphology. Additional sections of paraffin embedded tissue were used for immunohistochemical staining, which was performed on an automated immunostainer (Dako, Carpinteria, CA) using an avidin– biotin complex technique. Antibodies against the antigens CD20, CD79a, CD3 (Dako),
Fig. 2. Intrathoracic view of the right atrium. The tumor (arrow, tan mass) is seen superiorly, and the thin wall of the distended right atrial appendage reveals a hemorrhagic mass.
4. Pathologic findings The lesion was a multilobulated mass of smooth tissue measuring 6.5 cm in maximum dimension with a hemorrhagic cut surface (Fig. 3). Histology showed a diffuse proliferation of medium-sized immature lymphoid cells with a high nuclear/cytoplasmic ratio, an immature chromatin pattern, and inconspicuous nucleoli (Fig. 4). Brisk mitotic activity was seen with focal starry sky appearance. The immature lymphoid cells infiltrated the myocardial tissue and stained diffusely with the antigens CD79a, CD10, and
Fig. 4. Diffuse proliferation of lymphoblastic cells (H&E, 60).
118
D. Bassi et al. / Cardiovascular Pathology 13 (2004) 116–119
Fig. 5. Lymphoblasts are diffusely staining with anti-CD79a (immunoperoxidase, 40).
Fig. 7. Numerous lymphoblasts are positive for the nuclear antigen TdT (immunoperoxidase, 40).
TdT (Figs. 5– 7) and were negative for the CD20 antigen. Similarly, flow cytometric analysis revealed a CD45 dim population of immature lymphoid cells positive for the antigens CD19, cytoplasmic CD22 and CD79a, CD10, and nuclear TdT. The cells were negative for the CD20 antigen and did not express surface light chain immunoglobulins. The morphology and immunophenotype of the neoplastic cells were diagnostic of precursor B-LBL. Examination of the bone marrow and peripheral blood showed no evidence of precursor B lymphoblastic leukemia/lymphoma.
and hamartomas [4,5]. Malignant primary cardiac tumors are a minority, most of which represent various sarcomas. PCL is very rare in immunocompetent patients, comprising approximately 2% of all primary cardiac neoplasms and less than 0.5% of all extranodal lymphomas [2,4]. On the other hand, secondary involvement of the heart by malignant non-Hodgkin’s lymphoma is not uncommon and can be found in 15 – 30% of autopsy series [1]. PCL has been encountered with increasing frequency in patients with AIDS or other severe immunodeficiency states. The vast majority of cases are reported in adult and elderly patients, with slight predominance in males [2]. Chest pain and dyspnea are the most common presenting symptoms. Most lesions are solitary intracavitary tumoral masses involving the right atrium, majority of which represent DLBCL [2]. PCL in children is exceedingly rare. Our review of the English literature revealed few case reports of cardiac lymphoma in children, arising or presenting primarily in the heart (Table 1). However, the exact type and lineage of lymphoma and the extent of disease at presentation were not well documented in all cases. To the best of our knowledge, three cases of ‘‘cardiac lymphoblastic lymphoma’’ were previously reported in the English literature. One case was reported in a 13-year-old girl and was apparently of precursor B cell type [6]. Similar to our case, the pedunculated tumor mass arose from the right atrial wall, occupied the right atrium, and protruded into the right ventricle. However, this case did not fulfill the generally accepted strict definition of PCL since at presentation there was clinical evidence of disease in multiple extracardiac sites. The second case of ‘‘non-Hodgkin’s lymphoma of lymphoblastic type’’ involving the heart was reported in an 11-year-old boy [7]. In this case, the exact phenotype of the lymphoma (precursor B vs. T) was not documented. The hard and nodular tumor mass infiltrated the right atrial wall, extending through the pericardium to the right hilum. A third case of cardiac B-LBL was reported in an abstract form [11] in a 12-year-old boy. Here,
5. Discussion Primary cardiac tumors are rare and most are benign neoplasms. Myxomas are the most common and the rest include fibromas, lipomatous tumors, fibroelastic papillomas,
Fig. 6. Lymphoblasts are diffusely staining with anti-CD10 (immunoperoxidase, 40).
D. Bassi et al. / Cardiovascular Pathology 13 (2004) 116–119
119
Table 1 Summary of clinical and pathologic characteristics of cases of pediatric PCL reported in the literature and the current case Case no.
Age (years)/sex
Lymphoma type
Location
Outcome
Reference
1 2
13/female 13/male
Heart, right cheek, palate, and distal tibia ‘‘Left atrium’’
Relapsed Complete remission
[6] [8]
3 4
9/male 11/male
B-LBL ‘‘Small, noncleaved cell lymphoma’’ Burkitt lymphoma ‘‘Lymphoblastic lymphoma’’
Heart Right atrial mass extending through the pericardium into the hilum ‘‘Tumor infiltrating the right atrium, right ventricle, and lateral side of the left ventricle’’ Right and left atria, right suprahilar lymph nodes, pericardial and pleural effusions, and two areas of soft tissue in left chest wall Mass around the diaphragm involving the right heart border
Complete remission Dead
[2] [7]
Dead
[9]
In remission
[11]
In remission
[10]
Right intracavitary mass
Complete remission
Index case
5
8/?
‘‘Malignant non-Hodgkin’s lymphoma, type B’’
6
12/male
B cell lymphoblastic lymphoma
7
22 mo/female
8
10/male
B cell polymorphic post transplant lymphoma and recurrence (EBV positive ) B-LBL
three mass lesions were described: two in the right atrium and one in the left atrium. In addition, however, prominent right suprahilar lymph node enlargement, two nodules in the soft tissues of the left chest wall, and pericardial and pleural effusions were all described at presentation. Again, this case did not fulfill the strict definition of PCL. Other cases of PCL reported in children included a case of primary cardiac Burkitt’s lymphoma in a 9-year-old boy [2], a case of ‘‘small, noncleaved cell lymphoma’’ in a 13year-old boy [8], and a case of ‘‘highly malignant nonHodgkin’s lymphoma’’ in an 8-year-old child [9]. A case of polymorphic B cell posttransplant lymphoproliferative disorder developing around the diaphragm and secondarily involving the heart was also reported [10]. In our case, the intracavitary tumor mass was confined to the heart and showed immunomorphology diagnostic of a precursor B-LBL. There was no evidence of disease at any extracardiac site. Therefore, this is probably the first welldocumented case in a child. Histogenesis of PCL in immunocompetent patients is still unknown. There are different possible explanations for how PCL develop. Lymphomas can arise virtually in any extranodal site. In certain extranodal tissues, lymphomas arise from acquired extranodal lymphoid tissue after chronic inflammation and through a transformation process. However, association with a chronic inflammatory process and development of an acquired extranodal lymphoid tissue has not been well demonstrated in PCL. Alternatively, PCL may represent a metastasis or seeding from an occult and clinically undetectable lymphoma in a remote location from the heart. Further studies are clearly needed to better understand the histogenesis of PCL. In conclusion, PCLs are exceedingly rare in children and difficult to diagnose due to nonspecific clinical manifesta-
tions. This report shows that PCL should be considered in the differential diagnosis and work up of a child with cardiac neoplasm and that precursor B-LBL, a potentially curable disease, can present in a very unusual manner and site.
References [1] Burke A, Virmani R. Atlas of tumor pathology: tumors of the heart and great vessels. Third series. Fascicle 16. Washington, DC: Armed forces Institute of Pathology, 1996. pp. 1 – 11, 171 – 7. [2] Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier J-F, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of literature. Virchows Arch 2002;441:456 – 61. [3] Jaffe ES, Harris NL, Stein H, Vardiman JW. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Precursor B lymphoblastic leukemia/ lymphoblastic lymphoma. Tumors of hematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2001. pp. 109 – 11. [4] Fernandes F, Soufen HN, Ianni BM, Arteaga E, Ramires FJ, Mady C. Primary neoplasms of the heart. Clinical and histological presentation of 50 cases. Arq Bras Cardiol 2001;76(3):231 – 7. [5] Tazelaar HD, Locke TJ, Mc Gregor CGA. Pathology of surgically excised primary cardiac tumors. Mayo Clinic Proc 1992;67:957 – 65. [6] Kuo TT, Yang CP, Lin CH, Chang CH. Lymphoblastic lymphoma presenting as a huge intracavitary cardiac tumor causing heart failure. Pediatr Pathol 1987;7:341 – 9. [7] Rafeeq B, Narayandas K, Aboobacker CM. Non Hodgkin’s lymphoma presenting as cardiac tumor. Indian Pediatr 2001;38:543 – 5. [8] Moore JA, De Ran BP, Minor R, Arthur J, Fraker TD. Transesophageal echocardiographic evaluation of intracardiac lymphoma. Am Heart J 1992;124:514 – 6. [9] Ztot S, Cherradi R, Haddour L, Belhaj S, Kettani F, Benmimoun EG, Arharbi M. Primary cardiac lymphoma. Report of a case. Arch Mal Coeur Vaiss 2002;95(1):61 – 4. [10] Agarwala B, Rubin CM. Intracardiac lymphoma in a child; successful treatment with chemotherapy alone. Pediatr Cardiol 2000;21:401 – 2. [11] Das P, Caucao M, Weitzman S, et al. J Pediatr Hematol Oncol 2003; 25(4):511.