An unprecedented case report of primary cardiac lymphoma exclusive to left ventricle: a diagnostic and therapeutic challenge

Abstract

Introduction Primary cardiac lymphoma accounts for <2% of all primary cardiac tumours. It is uncommon in immunocompetent patients, often fatal and diagnosed at autopsy. Tumour usually involves the right heart chambers and pericardium. With advances in imaging, early diagnosis is possible and treatment including chemotherapy and surgery affords good prognosis. Case presentation We present a 50-year-old woman with abdominal pain and fevers for 5 days. Computed tomography of the abdomen showed splenic and renal infarcts but no mass or vegetation was noted on echocardiography. Thoracic computed tomography divulged a large left ventricular filling defect. Cardiac magnetic resonance imaging delineated a 3.5 × 4.5 cm anterobasal mass with frond-like projections and endocardial invasion without extracardiac involvement suggestive of a low-vascularity tumour. Echo-guided endomyocardial biopsy and minithoracotomy with needle biopsy were inconclusive. A sarcoid-protocol cardiac positron emission tomography-fluorodeoxyglucose scan showed focally elevated uptake in the basal anteroseptum without extracardiac uptake, supporting a malignant entity. This prompted open heart mass resection. Pathology revealed diffuse large B-cell lymphoma. Discussion Our case is a unique report of cardiac lymphoma isolated to the left ventricle. Location of the tumour and lack of specific imaging characteristics made it a diagnostic challenge. It underscores the importance of including lymphoma in the differential for intracardiac masses as it is responsive to chemotherapy. Additionally, it emphasizes the complementary role of imaging modalities and multidisciplinary team approach in diagnosis. Early diagnosis and therapy is the key to establishing successful outcomes.

Primary cardiac lymphomas may present under different phenotypes

“…[1][2][3] Due to obvious restrictions, not all can be referred to here. 4,5 A pedunculated, polylobulated non-infiltrative mass like that in the second case is a very uncommon presentation in an otherwise rare primary cardiac malignancy. This reinforces the fundamental value of histopathological and immunohistochemical examination of surgical specimens when dealing with intracardiac masses.…”

Immunomodulatory Treatment Strategies Targeting B Cells for Heart Failure

“…Data from histological analysis of autopsies indicate that B cells are present at a frequency similar to that of CD4 + and CD8 + T cells in normal human hearts (Noutsias et al, 2002). Moreover, the presence of B cells in the human heart is also supported by some rare case reports of primary cardiac B cell lymphoma (Grantomo et al, 2018;Thiagaraj et al, 2018;Li et al, 2019). In human specimens, B cells have also been discovered within the pericardial fat, with a relative increase in the size of pericardial-fat-associated lymphoid clusters in patients with coronary artery disease (Horckmans et al, 2018).…”

COUNTERPOINT: Should Isolated Cardiac Sarcoidosis Be Considered a Significant Manifestation of Sarcoidosis? No

“…Regarding alternative missed diagnoses, these include giant cell myocarditis, 10 other forms of myocarditis, 11 and primary cardiac lymphoma. 12 These have all been described to have similar 18 F-FDG PET scan appearances to "imaging isolated CS" (Fig 3).…”

“…IVS ¼ interventricular septum; LV ¼ left ventricle; RV ¼ right ventricle. (Reproduced with permission from Thiagaraj et al 12 ). H-J, 51-year-old woman presented with atypical chest pain, pre-syncope, and an elevated troponin.…”