Primary Cardiac Lymphoma: Importance of Tissue Diagnosis

Abstract

Primary cardiac lymphoma (PCL) is a rare disease entity that can present with severe cardiac and cardioembolic symptoms. We present a 79-year-old male with history of polymalgia rheumatica on chronic prednisone who presented with a two-week history of progressively worsening dyspnea, cough, and a 10 pound weight loss. Transthoracic echocardiogram (TTE) and computed tomography (CT) of the chest showed a large mediastinal mass with invasion of the pericardium. A biopsy of an abdominal soft-tissue mass confirmed the diagnosis of PCL. The patient was treated with two cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) which was complicated by progressive heart failure requiring substitution of liposomal doxorubicin. The epidemiology, presentation, diagnosis, and treatment options of PCL are discussed.

Resolution of HCM phenotype and CHF following CMOP protocol in a cat with large cell lymphoma and suspected cardiac involvement

“…3 Cardiac neoplasia is rare and has been estimated to represent <3% of all diagnosed tumours in dogs and humans. 1,5 In extensive human and canine cohort studies, PCL represents between 0.0017 and 0.19% of all diagnosed tumours, with MCL seen 20-40 times more often than PCL. 19,20 Evidence in cats is scant, with some reports suggesting that cardiac lymphoma is the most common cause of primary and metastatic neoplasia in the heart, with PCL comprising just 0.03% of cardiac cases.…”

“…24 Regardless of the advances in methods of cardiac biopsy, a study in humans found that only 200 out of 1,000,000 post-mortem cardiac masses were malignant, with the remaining being benign masses, often resulting in a reluctance to biopsy in species that can tolerate this procedure. 1,2 However, in cats, a definitive diagnosis is usually only obtained on post-mortem histopathology as feline jugulars cannot often support introducer catheters for endovascular biopsies, and due to the  of  suspected inherent risks of trans-thoracic/abdominal biopsies, these tests are rarely performed. 11,[25][26][27] However, a recent case report in which 25 gauge fine needle aspirates were performed on feline myocardium during pericardiectomy did not report any significant bleeding or arrythmias, suggesting this procedure maybe less high risk than originally suspected.…”

“…At the time of publishing, our patient was still in remission 21 months after the initial diagnosis, which is far more than the reported survival times in the current literature. 1,4,7,[9][10][11]16,20,40 There are several limitations to this study. The most significant limitation is the lack of cytology and histology confirming our hypothesis of cardiac lymphoma and excluding other differentials for TMT.…”

“…Lymphoma comprises one-third of feline cancers, with primary cardiac lymphomas (PCLs) and metastatic cardiac lymphomas (MCLs) being a rare and underdiagnosed group of lymphoid cancers. 1,2 This article presents a case of large cell splenic lymphoma with hypertrophic cardiomyopathy (HCM) phenotype, presumptively secondary to cardiac involvement of lymphoma. The patient was treated for congestive heart failure (CHF) and started soon after on a modified Madison-Wisconsin protocol (CMOP), resulting in the remission of the splenic lymphoma and resolution of HCM phenotype.…”

A Long Journey to the Truth: Primary Cardiac Lymphoma with Various Arrhythmias from Ventricular Tachycardia to Atrial Flutter

“…1) PCL has no pathognomonic presentation, manifested by location. Intramural tumors can present with fatal arrhythmia such as VT or conduction defects [2][3][4] at the beginning by invading the conduction system even before the structural lesion is detected. Continuous questions of VT mechanism and constant biopsies finally reached the proper diagnosis.…”