We present a case of a 70 year-old male with B-cell lymphoma of which the first clinical presentation was cardiac infiltration.
The patient underwent full chemotherapy with complete tumour regression.
Kardiol Pol 2011; 69, 10: 1063&#8211;106

Adamczyk, Tomasz

Gieszczyk, Klaudia

Grzywocz, Patryk

Gąsior, Zbigniew

Haberka, Maciej

Januszewski, Krzysztof

Mizia-Malarz, Agnieszka

Mizia-Stec, Katarzyna

Poborski, Wojciech

Via Medica Journals

www.kardiologiapolska.plOpis przypadku/Case report Kardiologia Polska2011; 69, 10: 1063–1065ISSN 0022–9032B−cell lymphoma in a 70 year−old male patientwith a cardiac tumourChłoniak B-komórkowy u 70-letniego mężczyzny z guzem sercaKatarzyna Mizia−Stec1, Maciej Haberka1, Patryk Grzywocz1, Krzysztof Januszewski2, Agnieszka Mizia−Malarz3,Tomasz Adamczyk1, Klaudia Gieszczyk1, Wojciech Poborski4, Zbigniew Gąsior11Department of Cardiology, Medical University of Silesia, Upper Silesian Medical Centre, Katowice, Poland2Histopathology Department, Medical University of Silesia, Katowice, Poland3Department of Oncology, Haematology and Chemotherapy, Medical University of Silesia,Upper Silesian Centre of Children’s Health Care, Katowice, Poland4Department of Oncology and Chemotherapy, District Hospital, Katowice, PolandA b s t r a c tWe present a case of a 70 year-old male with B-cell lymphoma of which the first clinical presentation was cardiac infiltration.The patient underwent full chemotherapy with complete tumour regression.Key words: B-cell lymphoma, cardiac infiltrationKardiol Pol 2011; 69, 10: 1063–1065Address for correspondence:prof. Katarzyna Mizia-Stec, Department of Cardiology, Medical University of Silesia, ul. Ziołowa 45/47, 40–635 Katowice, Poland,tel/fax: + 48 32 25 27 407, e-mail: kmizia@op.plReceived: 12.08.2010 Accepted: 15.09.2010Copyright © Polskie Towarzystwo KardiologiczneCASE REPORTA 70 year-old patient was admitted to our department witha three month history of exercise intolerance and dyspnoea(NYHA III), primarily associated with combined aortic valvedisease. The patient had never been hospitalised and was ful-ly physically active before these complaints occurred. Familyhistory was negative. At admission: good general condition,except for jugular veins distension and bradycardia, no otherpathological signs. The ECG revealed a third degree atrioven-tricular (III° A-V) block with an escape junctional rhythm (HR= 50/min). Transthoracic (TTE) and transoesophageal (TEE)echocardiography confirmed a moderate aortic valve steno-sis with massive valvular calcifications, hyperkinetic, non-en-larged left ventricle and excessive pericardial fluid (30 mmlayer). Pericardiocentesis was performed and 790 mL of dark--yellow exudative fluid was drained. Coronary angiographyrevealed the critical lesion within the second segment of rightcoronary artery — primary coronary angioplasty with a baremetal stent implantation was performed (TIMI 3). However,a III° A-V block persisted, which prompted us to implanta DDDR pacemaker. Pericardial fluid cytology, received on thethird day after pericardiocentesis, revealed Burkitt-like cells.Laboratory blood tests showed high erythrocyte sedimenta-tion rate (ESR) (53/100 mm/h) and low lipid parameters.A 64-multislice computer tomography (MSCT) of thechest revealed a left atrium posterior wall (thickness up to2 cm) and inter-atrial septum (IAS) (thickness up to 3 cm) neo-plasm infiltration with a density of 70 HU (soft tissue) — nofluid in pleura or pericardium (Fig. 1). Further imaging testsshowed no other primary or secondary neoplastic infiltrations.The recent pacemaker implantation and coronary angiopla-sty procedure motivated us to withdraw from the tumour bio-psy. Afterwards, the patient was discharged in a good andstable general condition with a recommendation of hospitali-sation within four weeks.At second admission, the patient was complaining of si-gnificant fatigue, apathy, and weight loss (5 kg in eight we-eks). There were no clinical signs of circulatory failure. Physi-cal examination revealed supraclavicular and axillar lympha-denopathy (3 cm). Control TTE demonstrated blurrily sepa-1064www.kardiologiapolska.plKatarzyna Mizia-Stec et al.rated tumour, localised within right atrium, right ventricle,along IAS and inter-ventricular septum (IVS) approximately57 ¥ 22 mm in dimensions (Fig. 2) and unaffected haemo-dynamic cardiac function. The 64-MSCT and ultrasonogra-phy showed several lymph nodes (up to 20 mm) in mediasti-num and only a single lymph node (17 mm) of extra-thoraciclocalisation, at the pancreas head area. Laboratory blood te-sts revealed increased ESR (80 mm/h), CRP (86.2 mg/L) andLDH (2614 U/L). The supraclavicular lymph node was takenfor histopathological examination (Fig. 3): the primary dia-gnosis was lymphoma malignum. The immunohistochemi-cal examination results were: CD20(+), CD3(+), CD43(+),CD79alpha(+), CD68(+), Bcl-2(+), Ki67>95%, TdT(–). Themolecular studies revealed MYC translocation.Finally, a diagnosis of B-cell lymphoma unclassifiablewith features intermediate between diffuse large B-cell lym-phoma (DLBCL) and Burkitt lymphoma (BL) was established.No pathology was found in the dermatomuscular biopsy.Afterwards, chemotherapy was administered (CHOP: cyclo-phosphamide, doxorubicin, vincristine, prednisone). TheTTE examination performed just after the first cycle of treat-ment revealed an impressive regression of neoplastic heartinfiltration limited only to the part of IAS (Fig. 4A). The pa-tient was in a good general clinical condition with onlya single axillary lymph node palpable. Further observationrevealed complete regression of the cardiac tumour (Fig. 4B),Figure 1. Scan of 64-multislice computer tomography;infiltration of the left atrial posterior wall and inter-atrialseptum (arrow)Figure 2. Transthoracic echocardiography — tumour localisedwithin the right atrium and the right ventricle along inter-atrialseptum; A. Four-chamber view; B. Zoom scan — tumour andpacemaker electrodeFigure 3. Histopathological examination of  the supraclavicularlymph node: 1) effacement of correct lymph node structure;2) extensive infiltration of mononuclear, middle size, little bitpolymorphic, round shaped or angular with macrogranularchromatin and not quite visible nucleoli; cytoplasm moderatelyabundant, basophilic, partially with plasmocyte-like pattern;numerous division figures, intercellular nucleus debris, apoptoticbodies, microgranular necrosis; 3) diffused macrophages withbright cytoplasm giving the ‘starry sky’ pattern (magnification¥ 350, and reproduced here at 70%)www.kardiologiapolska.pl1065B-cell lymphoma and a heart tumourwhich was also confirmed during aortic valve replacementcardiosurgery performed two years after the chemotherapy.DISCUSSIONThe reported case and the sequence of clinical findings stron-gly suggest that cardiac infiltration was the first presentationof BL and preceded peripheral lymphadenopathy. Pericar-dial effusion with atypical cells and a III° A-V block were pro-bably the first clinical signs of the disease. It is worth notingthat while initial TTE and TEE revealed no structural abnor-malities, it was the 64-MSCT examination performed due topericardial fluid cytology that showed the infiltrative structu-re within the atria.Available case reports have described incidentally foundprimary malignant heart lymphomas confirmed after its re-section [1–5]. Primary cardiac non-Hodgkin’s lymphomas areextremely rare and occur mainly in immunocompromisedpatients [6]. Of 35 cases of primary cardiac non-Hodgkin’slymphoma reported [7], 22 of them had DLBCLs.Figure 4. Transthoracic echocardiography, four-chamber view;A. Zoom scan; significant regression of the tumour limited tothe inter-atrial septum (arrow) after the first cycle of treatment;B. Complete echocardiographical regression of the heart tumourIn the presented case, tumour biopsy was temporarilydelayed (double antiplatelet treatment, implanted stent). Thenature of pathology (benign, malignant) was unknown, whichconvinced us that continuation of diagnostic procedures andpossible cardiosurgical intervention would be the optimalsolution. According to the recent update of the WHO Classi-fication of Tumours of Haematopoietic and Lymphoid Tissu-es [8], an eventual diagnosis of B-cell lymphoma unclassifia-ble with features intermediate between DLBCL and BL wasestablished. Several factors suggested the diagnosis of intra-vascular large B-cell lymphoma: advanced age, potentially pri-mary heart tumour, short medical history, aggressive course,and initial histopathological lymph node evaluation. How-ever, there were no skin abnormalities, or infiltration of centralnervous system and the dermatomuscular biopsy did not re-veal pathology. Moreover, high percentage of active cells inthe lymph node examined — Ki67>95% did not support thediagnosis of DLBCL [8, 9].We cannot unequivocally exclude a potential coincidenceof lymphoma and another heart tumour. However, the sequen-ce of presented events suggests the primary heart localisation.According to the available evidence based on case reports, onlyone third of patients survive longer than one year [10]. Our pa-tient underwent all required chemotherapy cycles and furtherclinical observation revealed complete tumour regression.Conflict of interest: none declaredReferences1. Alzeerah MA, Singh R, Jarrous A. Large B-cell lymphoma of theatria. Tex Heart Inst J, 2003; 30: 74–75.2. Boccardi L, Pino PG. Primary lymphoma of the right atrium:a case report. Ital Heart J Suppl, 2004; 5: 487–491.3. Timoteo AT, Gouveia R, Goncalves PA et al. Lymphoma withclinical presentation of a primary pericardial tumor. Rev PortCardiol, 2003; 22: 1385–1391.4. Igawa T, Nagafuji K, Ejima J et al. Surgical resection of malig-nant lymphoma in the right atrium after systemic chemothera-py. Intern Med, 2003; 42: 336–339.5. Cohen Y, Daas N, Libster D, Gillonb D, Polliack A. Large B-celllymphoma manifesting as an invasive cardiac mass: sustainedlocal remission after combination of methotrexate and rituximab.Leuk Lymph, 2002; 43: 1485–1487.6. Cahoon KP, Howe W, Mc Kiernan T. Primary cardiac diffuse,large B-cell lymphoma in an immunocompetent patient. J Inva-sive Cardiol, 2008; 20: E59–E60.7. Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier JF,Thivolet-Bejui F. Primary cardiac lymphoma in immunocom-petent patients: a report of three cases and review of the litera-ture. Virchows Arch, 2002; 441: 456–461.8. Swerdlow SH, Campo E, Harris NL et al. WHO Classification of Tu-mours of Haemopoietic and Lymphoid Tissues. 4th Ed., WHO 2008.9. Ponzoni M, Ferreri AJM, Campo E et al. Definition, diagnosis,and management of intravascular large B-cell lymphoma: pro-posals and perspectives from an international consensus meet-ing. J Clin Oncol, 2007; 25: 3168–3173.10. Cassidy J, Bissett D, Spense Obe RAJ. Haematological malig-nancies. In: Cassidy J, Bissett D, Spense Obe RAJ eds. OxfordHandbook of Clinical Oncology. Oxford University Press Inc,New York, 2002: 489–515.

Chłoniak B-komórkowy u 70-letniego mężczyzny z guzem serca

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Chłoniak B-komórkowy u 70-letniego mężczyzny z guzem serca

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