VIDEO CME


https://doi.org/10.5005/jp-journals-10089-0091
Journal of Acute Care
Volume 2 | Issue 3 | Year 2023

High-grade Non-Hodgkin’s Lymphoma Presenting as a Cardiac Tumor


Mohammed R Sayeed1, Somaraja Kamsalli2, Manjunath Boraiah3, Pradeep Rangappa4https://orcid.org/0000-0002-2187-8950, Karthik Rao5, Ipe Jacob6, Rajesh V Helavar7

1,2Department of Cardiovascular and Thoracic Surgery, Manipal Hospital Yeshwanthpur, Bengaluru, Karnataka, India

3–6Department of Critical Care, Manipal Hospital Yeshwanthpur, Bengaluru, Karnataka, India

7Department of Interventional Radiology, Manipal Hospital, Yeshwanthpur, Bengaluru, Karnataka, India

Corresponding Author: Ipe Jacob, Department of Critical Care, Manipal Hospital Yeshwanthpur, Bengaluru, Karnataka, India, Phone: +91 9844207268, e-mail: ipe.jacob@gmail.com

Received: 12 December 2023; Accepted: 01 January 2024; Published on: 19 February 2024

ABSTRACT

Non-Hodgkin’s lymphoma (NHL) is a malignancy of the lymphoid tissues, involving B cells, T cells, or natural killer cells. Primary cardiac lymphomas originating in the heart are very rare, accounting for <1% of all extranodal lymphomas. Extranodal spread to the heart resulting in a secondary cardiac lymphoma may be seen in 9–24% of patients with lymphoma. Patients may present with B symptoms such as fever, fatigue, night sweats, and weight loss, which may mask symptoms of cardiac involvement. Transesophageal echocardiography may be considered the imaging technique of choice but the definitive diagnosis requires histopathological confirmation. The following report describes the incidental finding of a high-grade NHL of the right atrium (RA).

How to cite this article: Sayeed MR, Kamsalli S, Boraiah M, et al. High-grade Non-Hodgkin’s Lymphoma Presenting as a Cardiac Tumor. J Acute Care 2023;2(3):161–162.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.

Keywords: Extranodal metastasis of non-Hodgkin’s lymphoma, Non-Hodgkin’s lymphoma, Primary cardiac tumor, Right atrial tumor, Secondary cardiac lymphoma, Transesophageal echocardiography

CASE DESCRIPTION

The patient was a 43-year-old male, with known hypothyroid, who presented with a history of fever, productive cough, dyspnea, pain in the abdomen, and dysuria for three days. Examination showed a raised jugular venous pulse, bilateral pitting pedal edema, grade II pansystolic murmur, and crepitations on the right side. The electrocardiogram and chest roentgenogram were unremarkable. A routine transthoracic echocardiogram (Video 1) showed a large nonhomogeneous mass occupying the entire right atrium (RA), attached to the RA-inferior vena cava (IVC) junction, inferred to be a thrombus. Computed tomography (CT) abdomen showed a possible thrombus in the suprahepatic segment of IVC, and enlarged pelvic lymph nodes while the CT chest showed a soft tissue mass in the right side of the pericardial sac in the RA and moderate right pleural effusion (Fig. 1). A follow-up transesophageal echocardiogram (TEE) suggested a myxoma (Video 2) (Fig. 2). Ultrasound-guided biopsy of a left external iliac lymph node mass revealed a high-grade lymphoma. The right pleural effusion was drained, but he remained in respiratory distress, requiring intubation and mechanical ventilation. About 10 days following his admission, he underwent surgical excision of the tumor with RA reconstruction using a bovine patch. However, his condition continued to deteriorate, eventually succumbing to shock and multiorgan failure.

Fig. 1: Computed tomography (CT) scan of chest axial and sagittal views showing right atrial mass (labels A and C) and right pleural effusion (label B)

Fig. 2: Transesophageal echocardiogram showing large right atrial mass (label A), and right ventricle (label RV)

DISCUSSION

Lymphomas that originate in the heart, or affect only the heart, are known as primary cardiac lymphomas.1 They are extremely rare as the heart is devoid of lymphoid tissue, accounting for just 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas.2 Secondary cardiac lymphomas due to extranodal spread to the pericardium or myocardium are relatively more common, seen in 9–25% of lymphoma patients. They usually involve the RA, as in the present case, followed by the pericardium. Early diagnosis is difficult as they usually present with dyspnea, and constitutional symptoms such as fever, chills, sweats, weight loss, and chest pain.3 Cardiac-specific signs and symptoms include right-sided heart failure, precordial pain, arrhythmia, conduction disorders, pericardial effusion, and cardiac tamponade.

By definition, secondary cardiac lymphomas are in stage IV of the disease; hence, diagnosis is usually late, leading to a poor prognosis. TEE is the imaging method of choice when evaluating an intracardiac mass and also for obtaining biopsy samples. However, imaging modalities may not be accurate at differentiating lymphomas from more common cardiac tumors such as myxomas. Definitive diagnosis requires histopathological examination of a biopsy sample, either of the tumor itself, a lymph node, or of pleural/pericardial fluid. Laboratory features that may suggest poor outcomes include raised lactate dehydrogenase, β2-microglobulin, and hypoalbuminemia. In an analysis of 94 cases of NHL with biopsy-proven cardiac involvement, median survival was 3 months (range, 0–72).3 Secondary cardiac lymphomas as well as those who presented with heart failure had inferior outcomes. Chemotherapy may help with prolongation in median survival. There is no evidence of increased survival with surgical treatment of these tumors.2 Early diagnosis and aggressive treatment may prolong the life of these patients.

SUPPLEMENTARY DATA

The supplementary videos 1 to 2 are available online on the website of https://www.jacutecare.com/journalDetails/JAC

Video 1: Transthoracic echocardiogram four-chamber view with color Doppler.

Video 2: Transesophageal echocardiogram (TEE) four-chamber view showing large right atrial mass impeding filling of the right ventricle.

ORCID

Pradeep Rangappa https://orcid.org/0000-0002-2187-8950

REFERENCES

1. Zhao Y, Huang S, Ma C, et al. Clinical features of cardiac lymphoma: an analysis of 37 cases. J Int Med Res 2021;49(3):300060521999558. DOI: 10.1177/0300060521999558

2. Camacho AF, Hernández FH, Bodes RS, et al. Primary cardiac lymphoma: diagnosis by transjugular biopsy. Rev Esp Cardiol 2003;56(11):1141–1144. DOI: 10.1016/s0300-8932(03)77026-2

3. Gordon MJ, Danilova O, Spurgeon S, et al. Cardiac non-Hodgkin’s lymphoma: clinical characteristics and trends in survival. Eur J Haematol 2016;97(5):445–452. DOI: 10.1111/ejh.12751

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