Primary Cardiac Lymphoma in a Young and Immunocompetent Patient Diagnosed by Percutaneous Transvenous Biopsy

Primary cardiac lymphoma is a rare cardiac neoplasm that is typically found in older, immunocompromised patients. In this case, we report an immunocompetent, 46-year-old female that presented with shortness of breath and chest discomfort. Diagnosis of primary cardiac lymphoma was confirmed by way of percutaneous transvenous biopsy under transesophageal echocardiography and cardiac fluoroscopy guidance. Primary cardiac lymphoma is a rare cardiac neoplasm that is typically found in older, immunocompromised patients. In this case, we report an immunocompetent, 46-year-old female that presented with shortness of breath and chest discomfort. Diagnosis of primary cardiac lymphoma was confirmed by way of percutaneous transvenous biopsy under transesophageal echocardiography and cardiac fluoroscopy guidance. Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin lymphoma. It accounts for only 1% to 2% of primary cardiac tumors and 0.5% of extranodal non-Hodgkin lymphomas.1Chin JY Chung MH Kim JJ Lee JH Kim JH Maeng IH Jung SY Hwang HJ Lee JB Youn HJ. Extensive primary cardiac lymphoma diagnosed by percutaneous endomyocardial biopsy.J Cardiovasc Ultrasound. 2009; 17: 141-144Crossref PubMed Google Scholar The most common type of PCL is diffuse large B cell lymphoma, and it is typically located in the right chambers of the heart.2Csizmar CM Sachs Z Cayci Z Bu L Linden MA. Primary cardiac lymphoma: three case reports and a review of the literature.Open J Blood Dis. 2021; 11: 120-132Crossref PubMed Google Scholar Presenting symptoms are often nonspecific including heart failure, chest pain, shortness of breath, arrhythmias, or pericardial effusion.1Chin JY Chung MH Kim JJ Lee JH Kim JH Maeng IH Jung SY Hwang HJ Lee JB Youn HJ. Extensive primary cardiac lymphoma diagnosed by percutaneous endomyocardial biopsy.J Cardiovasc Ultrasound. 2009; 17: 141-144Crossref PubMed Google Scholar The median age at diagnosis is 63 years. Most patients are immunocompromised with a male predominance.3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar Because of the nonspecific symptoms and rarity of PCL, multiple imaging studies are often done before a diagnosis is made. Definitive diagnosis is generally made by way of tissue biopsy.2Csizmar CM Sachs Z Cayci Z Bu L Linden MA. Primary cardiac lymphoma: three case reports and a review of the literature.Open J Blood Dis. 2021; 11: 120-132Crossref PubMed Google Scholar Chemotherapy is the standard of treatment.4Coiffier B Lepage E Briere J Herbrecht R Tilly H Bouabdallah R Morel P Van Den Neste E Salles G Gaulard P Reyes F Lederlin P Gisselbrecht C. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma.N Engl J Med. 2002; 346: 235-242Crossref PubMed Scopus (4554) Google Scholar A 46-year-old female with a medical history of anxiety, depression, and migraines presented to the emergency department with shortness of breath and chest discomfort. A noncontrast computed tomography (CT) scan of the chest showed a large pericardial effusion. A pericardial window showed inflammatory cells with no evidence of carcinoma. Subsequently, a right atrial mass was seen on transesophageal echocardiography (TEE) (Figure 1). Cardiac magnetic resonance imaging revealed a large infiltrative mass centered on the free wall of the right atrium extending to surround the superior vena cava and into the epicardial fat, inferior wall of the right atrium, intra-atrial septum, and the inferior wall of the right ventricle. The mass was measured at 6.5 × 5.3 cm. Thereafter, the patient underwent a percutaneous transvenous biopsy (PTVB) under TEE and cardiac fluoroscopy guidance. Biopsy results revealed diffuse large B cell lymphoma (Figure 2). The tumor cells were positive for PAX5, CD20, focally positive for CD10, and had a Ki-67 of >90%. They were negative for P53, MYC, and CD30. The patient's baseline lactate dehydrogenase (LDH) was just over 3,500. A CT of the chest, abdomen, and pelvis done for staging purposes revealed a large cardiac mass and homogenous appearance of the liver, representing passive congestion secondary to the mass within the heart. A bone marrow biopsy did not show any malignant involvement. The patient was treated with DA-R-EPOCH (dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin). Five cycles of R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) were given after. After chemotherapy, transthoracic echocardiogram (TTE) showed complete resolution (Figure 3).Figure 2Biopsy and pathologic examination of the mass shows DLBCL. DLBCL = diffuse large B cell lymphoma.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3TTE after chemotherapy shows no right atrial mass. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.View Large Image Figure ViewerDownload Hi-res image Download (PPT) PCL is an extremely rare cardiac tumor. It accounts for only 1.3% of all cardiac tumors and 0.5% of extranodal non-Hodgkin lymphomas.1Chin JY Chung MH Kim JJ Lee JH Kim JH Maeng IH Jung SY Hwang HJ Lee JB Youn HJ. Extensive primary cardiac lymphoma diagnosed by percutaneous endomyocardial biopsy.J Cardiovasc Ultrasound. 2009; 17: 141-144Crossref PubMed Google Scholar A large review conducted by Petrich et al3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar analyzed 197 cases of PCL from 1949 to 2009. Of the patients included in the study, the male:female ratio was 1.94 and the median age was 63 years. Patients ranged from 9 to 90 years old, but only 5 were 17 years old or younger. Furthermore, most patients were immunocompromised.3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar However, our case highlights an immunocompetent female in a younger age range. The most common presenting symptom of PCL was dyspnea, which was found in 64% of patients. Other presenting symptoms included arrhythmia (56%), congestive heart failure (47%), constitutional complaints (26%), chest pain (24%), pericardial effusion (58%), tamponade (20%), peripheral edema (9%), and superior vena cava syndrome (SVC) (5%).3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar The right-sided cardiac was more commonly affected than the left heart in PCL, with 92% of patients having right atrium or right ventricle involvement. The order of chamber involvement from greatest to least was right atrium, right ventricle, left atrium, and left ventricle. In 41% of patients, the atrial septum was involved, and in 25% of patients the SVC was involved. Laboratory data was most remarkable for elevations in LDH, with 92% of patients reporting LDH above the normal range.3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar Diagnosis may be difficult because of nonspecific symptoms. A thorough workup often includes TTE and TEE, CT, and magnetic resonance imaging. For diagnosis of primary cardiac tumors, TEE is more sensitive than TTE.5Chen X Lin Y Wang Z. Primary cardiac lymphoma: a case report and review of literature.Int J Clin Exp Pathol. 2020; 13: 1745-1749PubMed Google Scholar Fluorodeoxyglucose positive emission tomography/CT is helpful for determining if a mass is malignant and confirming cardiac isolation. Cytology from the pericardial or pleural effusion often shows atypical lymphoid cells, a monomorphic population of large cells, or many dissociated small round cells. The specificity of cytology is low, and tissue biopsy is generally needed to make the diagnosis.6Spriggs AI Vanhegan RI. Cytological diagnosis of lymphoma in serous effusions.J Clin Pathol. 1981; 34: 1311-1325Crossref PubMed Google Scholar Historically, the biopsy approach has been by way of sternotomy with incisional biopsy, but this invasive procedure comes with high morbidity and mortality. PTVB with TEE carries a lower risk of complications.7Flox Camacho A Hernández Hernández F Salguero Bodes R Sánchez Pérez I Carbonell Porras A Tascón Pérez J Linfoma cardíaco primario: diagnóstico mediante biopsia transyugular [Primary cardiac lymphoma: diagnosis by transjugular biopsy].Rev Esp Cardiol. 2003; 56: 1141-1144Crossref PubMed Google Scholar A study examining various diagnostic options for PCL revealed that utilizing TEE-guided biopsy achieved a 100% success rate.8Ceresoli GL Ferreri AJ Bucci E Ripa C Ponzoni M Villa E. Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.Cancer. 1997; 80: 1497-1506Crossref PubMed Scopus (256) Google Scholar Furthermore, the frequency of complications because of PTVB are quite low, with studies reporting a range of 1.17% to 1.67%. Some of the more severe complications include cardiac perforation and tamponade. Others include arrhythmias, tricuspid regurgitation, coronary sinus disruption, interventricular septum perforation, and thrombus formation.9Savoia MT Liguori C Nahar T Marboe C Weinberger J Di Tullio MR Homma S. Transesophageal echocardiography-guided transvenous biopsy of a cardiac sarcoma.J Am Soc Echocardiogr. 1997; 10: 752-755Abstract Full Text Full Text PDF PubMed Google Scholar Complications of sternotomy are more severe, particularly sternal wound infections which may lead to surgical re-exploration, low cardiac output syndrome, and prolonged ventilation.10Grossi EA Culliford AT Krieger KH Kloth D Press R Baumann FG Spencer FC. A survey of 77 major infectious complications of median sternotomy: a review of 7,949 consecutive operative procedures.Ann Thorac Surg. 1985; 40: 214-223Abstract Full Text PDF PubMed Google Scholar The standard of treatment for PCL is chemotherapy, and complete remission has been achieved with R-CHOP. A study compared R-CHOP chemotherapy to CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) alone and found that adding rituximab was associated with a higher complete-response rate and overall survival, without a considerable increase in toxicity.4Coiffier B Lepage E Briere J Herbrecht R Tilly H Bouabdallah R Morel P Van Den Neste E Salles G Gaulard P Reyes F Lederlin P Gisselbrecht C. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma.N Engl J Med. 2002; 346: 235-242Crossref PubMed Scopus (4554) Google Scholar Surgical debulking before chemotherapy is only done in select patients such as those with SVC syndrome or rapidly progressive heart failure.2Csizmar CM Sachs Z Cayci Z Bu L Linden MA. Primary cardiac lymphoma: three case reports and a review of the literature.Open J Blood Dis. 2021; 11: 120-132Crossref PubMed Google Scholar According to Petrich et al,3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar complete response to treatment was found in 59% of patients. The median time to relapse was 7 months, and of the 197 patients, 12 showed relapse.3Petrich A Cho SI Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.Cancer. 2011; 117: 581-589Crossref PubMed Scopus (201) Google Scholar Thus, monitoring patients for relapse may be useful, but there are no current guidelines in place. In conclusion, PCL is a very rare neoplasm that is difficult to diagnose because of nonspecific symptoms. Historically, definitive diagnosis has required an invasive sternotomy. Diagnosis with PTVB carries fewer risks and may be a favorable alternative. This case highlights a rare cardiac neoplasm in an immunocompetent, 46-year-old female diagnosed through this method. The authors have no conflicts of interest to declare.