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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 591-593
Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype

1 Department of Pathology, Tata Memorial Hospital, Mumbai, India
2 Department of Radiodiagnosis, Tata Memorial Hospital, Mumbai, India
3 Department of Medical Oncology, Tata Memorial Hospital, Mumbai, India

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Date of Web Publication20-Sep-2011


Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.

Keywords: Cardiac tumor, diffuse large B-cell lymphoma, non-Hodgkin′s lymphoma

How to cite this article:
Gadage V, Kembhavi S, Kumar P, Shet T. Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype. Indian J Pathol Microbiol 2011;54:591-3

How to cite this URL:
Gadage V, Kembhavi S, Kumar P, Shet T. Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Nov 29];54:591-3. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/591/85104

   Introduction Top

Primary cardiac tumors are rare (autopsy incidence-0.056%) and the most common tumor involving the heart is metastasis (autopsy incidence-1.23%), mostly from lung and breast carcinoma. [1],[2] Secondary cardiac involvement in lymphoma patients is known in terminal stages and may occur in 10-20% of patients with lymphoma. [2],[3] Primary cardiac tumor (PCL) on other hand is an extremely rare and fatal disorder. Diffuse large B-cell lymphoma (DLBCL) is the commonest PCL. [3] PCL is usually an oncologic emergency and surgical debulking helps to relieve the obstructive symptoms but response to chemotherapy is gratifying. We report of case of PCL of DLBCL with immunophenotypic features, in a 38-year-old immunocompetent male who responded to chemotherapy and had 10-month survival after the diagnosis.

   Case Report Top

A 38-year-old male presented with features suggestive of superior vena cava (SVC) obstruction such as facial puffiness, dyspnoea and distended nonpulsatile jugular veins. Transesophageal echocardiography (TEE) confirmed SVC obstruction caused by a large cauliflower mass inside the right atrium extending into the right ventricle, and obstructing the right ventricular entry of the inferior vena cava (IVC). This mass also had extravascular extension. Pericardial effusion along with left pleural effusion was evident. Imaging studies (computed axial tomography (CT) scan and magnetic resonance imaging (MRI)) of the neck and chest revealed a right atrial irregular mass lesion [Figure 1], possibly a myxoma with thrombus involving the proximal portion of SVC, innominate and left jugular vein. The CT chest and abdomen at the time of presentation had shown small mediastinal lymph nodes with basal lung consolidation and pleural effusion. No significant lymphadenopathy was documented. In view of the deteriorating cardiac status, the right ventricular outflow obstruction was relieved by partial surgical resection of mass (with a provisional impression of myxoma) under TEE guidance.
Figure 1: A CT scan showing the mass filling the right atrial cavity

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Histopathological examination showed monotonous population of large discohesive cells with apoptosis and large areas of necrosis. Most cells had centroblastic morphology, however, a few areas had more pleomorphic cells with anaplastic nuclei [Figure 2]a. The tumor cells were CD45 and CD20-positive with a MIB1 index of 90%. BCL2 and MUM1 and FOXP1 were positive [Figure 2]b-d while the tumor cells were negative for CD3, CD10, BCL6, CD5, cyclin D1, SMA, S100, desmin, Tdt and EBER-ISH (Epstein Barr virus in situ hybridization (Novocastra kit)). The histopathology diagnosis offered was diffuse large B-cell lymphoma/DLBCL
Figure 2: (a) Tumor was composed of discohesive large centroblast-like cells with apoptosis and mitosis (hematoxylin and eosin/H and E: 400), (b) Diff use CD20 positivity within the tumor cells (avidin biotin complex/ABC: 400), (c) High MIB1 labeling index in the tumor (ABC: 200), (d) Nuclear MUM1 expression (ABC: 400)

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Staging bone marrow was uninvolved. Serology studies for human immunodeficiency virus (HIV), Hepatitis virus surface antigens (HBsAg)and Hepatitis C virus (HCV) were non-reactive. Chemotherapy with Rituximab (R) and CHOP regimen (six cycles) was planned. After four cycles of chemotherapy, the intra - atrial mass disappeared (confirmed on TEE and CT scan). However at the end of the sixth cycle of R-CHOP, his symptoms reappeared and on TEE study an intra - atrial recurrence of tumor mass measuring 1 Χ 4 cm, attached to the right side of the interatrial septum was documented. Findings were confirmed on CT scan of thorax which revealed an ill - defined residual soft tissue mass in the mediastinum in the precarinal and subcarinal region, infiltrating the aortopulmonary window, pericardium and left atrium with thrombosis of the right inferior pulmonary vein. At this time multiple lymphomatous deposits were seen in both kidneys and adrenals. He was started with Rituximab and DHAP (cisplatin in combination with high-dose Ara-C and dexamethasone) regimen. After completion of two cycles of this regimen, the patient developed chest pain, severe hemoptysis, and progressive disease, eventually succumbing to the complications thereof.

   Discussion Top

Currently the accepted definition of PCL is a lymphoma presenting as primary cardiac lesion with bulk of tumor within the pericardium or with cardiac symptoms from lymphomatous cardiac infiltration at the time of presentation. [1],[4] In the case we discuss the clinical presentation of SVC obstruction secondary to the right atrial mass with lack of significant lymphadenopathy and no marrow involvement confirmed the diagnosis of PCL.

PCL is extremely rare, accounting for 1.3% of the primary tumors of heart and 0.5% of extranodal lymphoma. [1] Disseminated lymphoma with cardiac involvement occurs in up to 10-20% of patients with lymphoma at the time of death. [2],[3] PCL occurs more commonly in immunocompromised patients and is extremely uncommon in immunocompetent patients. [1],[2],[5],[6] The median age of presentation of PCL was above 60 years [2],[3],[6] while the case we report is the youngest immunocompetent patient ever reported in the literature to the best of our knowledge.

PCL presents clinically in a variety of ways with no pathognomic signs or symptoms to lesions masquerading as cardiac disease. The most common presenting symptoms reported in PCL are refractory (right-sided) heart failure (52%), pericardial pain (17%), rhythm/Electrocardiogram abnormalities, cardiac tamponade (12%) and uncommonly with superior vena cava syndrome (8%) and myocardial involvement (0.5%). [1],[3],[6] Unusual presentations such as SVC syndrome have been reported only in two cases studied for PCL. [2],[5] Right atrial involvement is much more common as compared to cardiac tamponade in PCL. [2],[4],[6] This patient presented with SVC obstruction due to cardiac mass which is a life-threatening emergency.

Histological differential diagnoses of a poorly differentiated malignant neoplasm involving the heart include metastatic carcinoma, high-grade lymphoma, metastatic malignant melanoma, and undifferentiated sarcoma. All these may show diffuse large atypical cells with prominent nucleoli and mitotic activity of the cells and immunohistochemistry is essential for accurate diagnosis. Expression of CD45 and CD20-with lead to the diagnosis of DLBCL. DLBCL is the most common type of PCL constituiting about 80% of cases of PCL while Burkitt's lymphoma and T-cell lymphoma are in minority. [1],[2],[3],[4],[5],[6]

Gene profiling experiments have demonstrated aggressive behavior in a set of diffuse large B-cell lymphomas labeled as activated B cell (ABC) type. [7] A number of immunophenotypic surrogates have been proposed to identify the ABC or essentially postgerminal centre lymphomas. The most popular is the Hans algorithm based on immunostaining for CD10, Bcl-6, and MUM1/IRF4 with a misclassification rate of 20%, with first two being germinal centre markers and the latter a postgerminal centre marker. [8] In view of the variability of staining for CD10 and Bcl6 some authors have advocated the use of MUM1/IRF4 and FOXP1 to identify the aggressive ABC subtype of DLBCL. [9] DLBCL from different sites have been shown to have predominantly an ABC phenotype explaining their aggressive behavior. Though we discuss a single case only, expression of markers like FOXP1 and MUM1 prompts us to think that PCL may also have a ABC like phenotype explaining its aggressive behavior.

With better imaging modalities and access to cardiac biopsy, PCL is usually diagnosed antemortem. Accurate histopathological diagnosis of PCL is very important, as PCL is chemo-radiosensitive and with prompt treatment the survival of these patients can be improved. The choice of chemotherapy for PCL remains the Rituximab and CHOP regimen. [6],[10] The prognosis of PCL, however, continues to still be poor as also seen in the case we discussed and modified regimens are still underway for the treatment of this tumor. [10]

   References Top

1.McCallister HA, Fenoglio JJ. Tumors of the cardiology system, Atlas of tumour Pathology, 2nd series, fascicle 15. Washington, DC: Armed Forces Institute of Pathology; 1978. p. 99-100.  Back to cited text no. 1
2.Nascimento AF, Winters GL, Pinkus GS. Primary cardiac lymphoma: Clinical, histologic, immunophenotypic features of 5 cases of a rare disorder Am J Surg Pathol 2007;31:1344-50.  Back to cited text no. 2
3.Gowda RM, Khan IA. Clinical prospective of primary cardiac lymphoma. Angiology 2003;54:599-604  Back to cited text no. 3
4.Zaharia L, Gill PS. Primary cardiac lymphoma. Am J Clin Oncol 1991;14:142-4.  Back to cited text no. 4
5.Chalabreysse L, Berger F, Loire R, Devossoux G, Gordier JF, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients: A report of three cases and review of the literature. Virchow's Arch 2002;441:456-61.  Back to cited text no. 5
6.Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E. Primary cardiac lymphoma in immunocompetent patients, diagnostic and therapeutic management. Cancer 1997;80:1497-504.  Back to cited text no. 6
7.Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS, Rosenwald A, et al. Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling. Nature 2000;403:503-11.  Back to cited text no. 7
8.Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Blood 2004;103:275-82.  Back to cited text no. 8
9.Nyman H, Jerkeman M, Karjalainen-Lindsberg ML, Banham AH, Leppä S. Prognostic impact of activated B-cell focused classification in diffuse large B-cell lymphoma patients treated with R-CHOP. Mod Pathol 2009;22:1094-01.  Back to cited text no. 9
10.Dawson MA, Mariani J, Taylor A, Koulouris G, Avery S. The successful treatment of primary cardiac lymphoma with a dose dense schedule of Rituximab plus CHOP. Ann Oncol 2006;17:176-7.  Back to cited text no. 10

Correspondence Address:
Tanuja Shet
Department of Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Mumbai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85104

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