American Journal of Emergency Medicine 33 (2015) 1849.e5–1849.e6
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Rare cause of a common symptom: primary cardiac lymphoma☆,☆☆,★,★★
Abstract Cardiac tumors may be symptomatic or found incidentally during evaluation for a seemingly unrelated problem or physical finding. Because symptoms may mimic other cardiac conditions, the clinical challenge is to consider the possibility of a cardiac tumor so that the appropriate diagnostic test(s) can be conducted. In this case, we describe an exceedingly rare cardiac tumor. Primary cardiac lymphoma (PCL) is a rare tumor with nonspecific signs and symptoms that delay the diagnosis and management [1]. Primary cardiac lymphoma is found incidentally during evaluation for a seemingly unrelated problem. Primary cardiac tumors are extremely uncommon with all primary cardiac tumors of less than 5%, only 1.3 % is PCL [2,3]. Incidence is higher in immunocompromised hosts with B cell being the most common type. A 62-year-old man presented with a 9-month history of fatigue and shortness of breath with exertion, which was associated with night sweats and 20 lbs unintentional weight loss. His past medical history was significant for hypertension and coronary artery disease with cardiac bypass surgery 5 years before presentation. He denied chest pain, palpitation, or syncope. On arrival, vitals were stable, and physical exam was remarkable for elevated jugular venous pressure and cannon A wave. Chest x-ray showed mediastinal widening and cardiomegaly. Patient underwent echocardiogram that was significant for large left atrial mass obstructing the tricuspid valve and causing severe tricuspid stenosis with trivial tricuspid regurgitation and obstructing right ventricle outflow tract. This was followed by computed tomography scan of the chest that was significant for infiltrative cardiac lymphoma centered on the right atrioventricular groove with invasion of the pericardium, right atrium, tricuspid valve, interatrial septum, and right ventricle free wall. Positron emission tomography scan was done and showed enhancement to the interatrial area (Figs. 1 and 2). Right heart catheterization was done, and several biopsies of the lesion were taken. Pathology revealed diffuse large B-cell lymphoma. Patient was deemed inoperable at this stage because of cardiac extensive invasion of the myocardium and aortic root. Chemotherapy with the rituximab, cyclophosphamide, adriamycin, vincristin, prednisone (R-CHOP) was initiated. Initially, the tumor responded to the chemotherapy. Primary cardiac lymphomas are rare tumors with nonspecific signs and symptoms that delay the diagnosis and management. Approach to
☆ Financial support: None for all authors. ☆☆ Disclosure and contribution: All authors have no disclosures and had a role in writing the manuscript. ★ Funding source(s): None. ★★ Conflict of interest statement for all authors: NoneAll of the above listed authors had access to the manuscript and a role in writing the manuscript.
0735-6757/© 2015 Elsevier Inc. All rights reserved.
these cases should be conservative starting with echocardiogram. Most PCL tumors involve the right chambers and pericardium and usually aggressive with high morbidity and mortality [3]. Patients usually present with beside echocardiogram and computed tomography scan. Positron emission tomography scan is considered an important step for the evaluation of cardiac tumors by providing an efficient way to measure tumor metabolism and proliferation, examine anatomic structure [4], and help in identifying the appropriate biopsy sites and in monitoring treatment response. Biopsy is the criterion standard for the diagnosis of cardiac infiltrative diseases. Treatment is surgical if feasible and chemotherapy with the standard chemo combination (R-CHOP), which showed improved survival with the addition of rituximab to chemotherapy regimens [5]. Tissue necrosis near the heart and great vessel poses a great risk for perforation and death. The fragile tumor can also cause fatal pulmonary embolism [3,5,6], chest pain, pericardial effusion, cardiac arrhythmias, or symptomatic valvular heart disease [6]. In addition to obstructing circulation through the right side of the heart, tumor fragments may be released into the pulmonary circulation, causing symptoms consistent with pulmonary emboli [3,6]. Aiyah Jandali University of South Florida Medical School, Tampa, Fl Amjad Kabach, MD Department of Medicine, Creighton University, Omaha, NE 68131 Corresponding author. Department of Medicine, Creighton University Omaha, NE 68131 E-mail address:
[email protected] Shadi Al halabi, MD, MPH Department of Medicine, Cleveland Clinic, Cleveland, OH 44106 E-mail address:
[email protected] M. Chadi Alraies, MD Department of Medicine, Division of Cardiovascular Medicine, University of Minnesota, Minneapolis, MN 55455 E-mail address:
[email protected]
http://dx.doi.org/10.1016/j.ajem.2015.04.068 References [1] Anghel G, Zoli V, Petti N, Remotti D, Feccia M, Pino P, et al. Primary cardiac lymphoma: Report of two cases occurring in immunocompetent subjects. Leuk Lymphoma 2004; 45(4):781–8. [2] Ikeda H, Nakamura S, Nishimaki H, Masuda K, Takeo T, Kasai K, et al. Primary lymphoma of the heart: Case report and literature review. Pathol Int 2004;54(3):187–95. [3] Miguel CE, Bestetti RB. Primary cardiac lymphoma. Int J Cardiol 2011;149(3):358–63. [4] Buchmann I, Wandt H, Wahl A, Reske SN. FDG PET for imaging pericardial manifestations of Hodgkin lymphoma. Clin Nucl Med 2003;28(9):760–1.
1849.e6
A. Jandali et al. / American Journal of Emergency Medicine 33 (2015) 1849.e5–1849.e6
Fig. 1. A, Positron emission tomography/CT scan frontal plane with view of enlarged heart especially the left ventricle; B, increased uptake in the interatrial area with right ventricle enhancement; C, increase in metabolic activity in interatrial area with right ventricle.
[5] Nakagawa Y, Ikeda U, Hirose M, Ubukata S, Katsuki TA, Kaminishi Y, et al. Successful treatment of primary cardiac lymphoma with monoclonal CD20 antibody (rituximab). Circ J 2004;68(2):172–3. [6] Nascimento AF, Winters GL, Pinkus GS. Primary cardiac lymphoma: Clinical, histologic, immunophenotypic, and genotypic features of 5 cases of a rare disorder. Am J Surg Pathol 2007;31(9):1344–50.
Fig. 2. A, Positron emission tomography/CT scan horizontal plane with enlarged heart especially the left ventricle; B, increased uptake in interatrial area; C, increase in metabolic activity in interatrial area with right ventricle.
