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      Is Open Access

      Primary Cardiac Lymphoma Manifesting as an Atrioventricular Block in a Renal Transplantation Recipient

      case-report

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          Abstract

          Our report illustrates an atrioventricular block due to primary cardiac lymphoma attached to the right atrial septum that was rapidly reversible by surgical debulking and effective chemotherapy without the need for a permanent pacemaker. ( Level of Difficulty: Beginner.)

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          Abstract

          Our report illustrates an atrioventricular block due to primary cardiac lymphoma attached to the right atrial septum that was rapidly reversible…

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          Most cited references19

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          Differentiation of malignant and benign cardiac tumors using 18F-FDG PET/CT.

          Lars Stegger, Klaus Tiemann, Kambiz Rahbar … (2012)
          In the diagnostic algorithm of cardiac tumors, the noninvasive determination of malignancy and metastatic spread is of major interest to stratify patients and to select and monitor therapies. In the diagnostic work-up, morphologic imaging modalities such as echocardiography or magnetic resonance tomography offer information on, for example, size, invasiveness, and vascularization. However, preoperative assessment of malignancy may be unsatisfactory. The aim of this study was to evaluate the diagnostic value of (18)F-FDG PET and the incremental diagnostic value of an optimized CT score in this clinical scenario. (18)F-FDG PET/CT scans (whole-body imaging with low-dose CT) of 24 consecutive patients with newly diagnosed cardiac tumors were analyzed (11 men, 13 women; mean age ± SD, 59 ± 13 y). The maximum standardized uptake values (SUV(max)) of the tumors were measured. Patients were divided into 2 groups: benign cardiac tumors (n = 7) and malignant cardiac tumors (n = 17) (cardiac primaries [n = 8] and metastases [n = 9]). SUV(max) was compared between the 2 groups. Results were compared with contrast-enhanced CT, using standardized criteria of malignancy. Histology served as ground truth. Mean SUV(max) was 2.8 ± 0.6 in benign cardiac tumors and significantly higher both in malignant primary and in secondary cardiac tumors (8.0 ± 2.1 and 10.8 ± 4.9, P < 0.01). Malignancy was determined with a sensitivity of 100% and specificity of 86% (accuracy, 96%), after a cutoff with high sensitivity (SUV(max) of 3.5) was chosen to avoid false-negatives. Morphologic imaging reached a sensitivity of 82% and a specificity of 86% (accuracy, 83%). Both false-positive and false-negative decisions in morphology could be corrected in all but 1 case using a metabolic threshold with an SUV(max) of 3.5. In addition, extracardiac tumor manifestations were detected in 4 patients by whole-body (18)F-FDG PET/CT. (18)F-FDG PET/CT can aid the noninvasive preoperative determination of malignancy and may be helpful in detecting metastases of malignant cardiac tumors.
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            The 2015 WHO Classification of Tumors of the Heart and Pericardium.

            Fabio Tavora, Allen P Burke (2016)
            This article reviews the nomenclature of benign and malignant neoplasm of the heart and pericardium in the 4th edition of the World Health Organization's Classification, with emphasis on differences since the 3rd edition of 2004. The tumours are divided into benign, malignant, and intermediate tumors of uncertain behavior, with separate sections on germ cell tumours and tumors of the pericardium. There are important updates in the sarcoma classification, with emphasis on the most common site, the left atrium. The importance of the new genetic finding in cardiac myxomas, namely somatic mutations in the PRKAR1A gene underscores the importance of this alteration in the pathogenesis of these tumors. Challenges on the classification of each entity are discussed.
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              NCCN Guidelines Insights: B-Cell Lymphomas, Version 3.2019.

              Andrew D Zelenetz, Leo Gordon, Jeremy S. Abramson … (2019)
              Diffuse large B-cell lymphomas (DLBCLs) and follicular lymphoma (FL) are the most common subtypes of B-cell non-Hodgkin's lymphomas in adults. Histologic transformation of FL to DLBCL (TFL) occurs in approximately 15% of patients and is generally associated with a poor clinical outcome. Phosphatidylinositol 3-kinase (PI3K) inhibitors have shown promising results in the treatment of relapsed/refractory FL. CAR T-cell therapy (axicabtagene ciloleucel and tisagenlecleucel) has emerged as a novel treatment option for relapsed/refractory DLBCL and TFL. These NCCN Guidelines Insights highlight important updates to the NCCN Guidelines for B-Cell Lymphomas regarding the treatment of TFL and relapsed/refractory FL and DLBCL.
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                Author and article information

                Contributors
                Jung Myung Lee: @JungMyungLee2
                Journal
                Journal ID (nlm-ta): JACC Case Rep
                Journal ID (iso-abbrev): JACC Case Rep
                Title: JACC Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 2666-0849
                Publication date PMC-release: 15 April 2020
                Publication date Collection: April 2020
                Publication date (Electronic): 15 April 2020
                Volume: 2
                Issue: 4
                Pages: 600-603
                Affiliations
                [1]Division of Cardiology, Department of Internal Medicine, Kyung Hee University Medical College, Kyung Hee University, Seoul, Republic of Korea
                Author notes
                [∗ ] Address for correspondence: Dr. Jung Myung Lee, Division of Cardiology, Department of Internal Medicine, Kyung Hee University Medical College, Kyung Hee University, Hoegi-dong 1, Dongdaemun-gu, Seoul 130-701, Republic of Korea. cardioljm@ 123456khu.ac.kr @JungMyungLee2
                Article
                Publisher Item ID: S2666-0849(20)30147-9
                DOI: 10.1016/j.jaccas.2020.01.028
                PMC ID: 8298532
                PubMed ID: 34317303
                SO-VID: 633cf55b-1f2b-459d-b333-4454bcb8124c
                Copyright © © 2020 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 23 January 2020
                Date accepted : 29 January 2020
                Categories
                Subject: Mini-Focus Issue on Electrophysiology and Pacing
                Subject: Case Report: Clinical Case

                Keywords: cardiac mass,diffuse large b cell lymphoma,immunosuppressant,primary cardiac lymphoma,av, atrioventricular,ecg, electrocardiogram,pcl, primary cardiac lymphoma,pet-ct, positron emission tomography-computed tomography,pod, postoperative day,tte, transthoracic echocardiography
                Data availability:
                Keywords: cardiac mass, diffuse large b cell lymphoma, immunosuppressant, primary cardiac lymphoma, av, atrioventricular, ecg, electrocardiogram, pcl, primary cardiac lymphoma, pet-ct, positron emission tomography-computed tomography, pod, postoperative day, tte, transthoracic echocardiography

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