Primary Cardiac Burrkit Lymphoma: Characteristics, Clinical Presentation and Outcome

Abstract

Background:Primary cardiac lymphoma (PCL) is rare extranodal subtype of non-Hodgkin's lymphoma (NHL) .It is extremely rare in immunocompetent patients. PCL usually occurs in adults mean age of 62.1 years with male predominance. Primary cardiac Burkitts lymphoma (PCBL) is small subgroup of the PCL. Characteristics, presentation and the optimal management guideline of BL have not been clearly defined.Method:We performed PubMed searches using the particular terms including but not limited to primary, cardiac, burrkit's , lymphoma to identify reported adult cases of PCBL.Different Variables including demographic, immune status, clinical presentation, radiographic and other clinical variables were analyzed.Result:This is the largest study of PCBL up to now.We analyzed 10 cases of adult PCBL including 9 reported cases in literature and one case who presented to our facility.Majority of patients were older than 65 (7/10), which is consistent with other studies of Burrkit's lymphoma in immunocompetent patients particularly in sporadic type. This can be secondary to similarity of pathogenesis and oncogenesis of PCBL to sporadic type. Although age at presentation ranged from 30 to 85 years old, only (2/10) were younger than 40. .All reported PCBL in our analysis including the one who presented to our facility were HIV negative and immunocompetent state. Thus we propose that PCBL can be categorized as a subtype of sporadic BL (SBL) but interestingly PCBL was more common in older patients in contrast with SBL as is more common in younger individuals, with a peak incidence at age 30 in adults. Most of patients (7/10) were male, which is also consistent with other studies of majority of NHL subgroups including cardiac lymphoma. As expected all patients except one presented with cardiac symptoms. Dyspnea was the most prominent presenting symptom comparing to palpitation, syncope or chest pain. It is most likely secondary to large pericardial effusion, which was present in all patients, rather than the mass effect. Interestingly one case had no cardiac mass identified and PCBL was diagnosed with pericardial analysis. These demonstrate the importance of evaluation for and analysis of possible pericardial effusion in patients, who present with dyspneaAs noted, our analysis showed that all patient except the one who presented to our facility, presented with cardiac mass (9/10). Similar to other studies of cardiac lymphoma, right heart particularly right atrium was the most common location of the cardiac mass. Cardiac lymphoma should be highly considered in patient with right atrial mass.Interestingly, Outcomes and chemotherapy response were variable; 3/10 cases reported favorable response to chemotherapy , however two cases with variable burrkit type and other chromosomal abnormalities had poorer prognosis. Due to rarity and low number of the cases ,outcome analysis adjusted with treatment regimen was not performed in our study.Conclusion:There is no comprehensive treatment analysis study for burrkit lymphoma . Due to complexity of pathogenesis and different behavior of this type of lymphoma, an efficient and optimal diagnostic, treatment and management guidelines for Burrkit lymphoma and its subtypes need to be developed.Table 1AgeGender(Male : M, Female : F)Presenting SymptomMass Locationpericardial effusionDiagnosed byChromosomal abnormalitiesHIV statusReference79FDyspneaLeft Ventricle (LV)Positive (pos)Pericardial analysisvariant type-(8,22)(q24,q11), no c-mycnegative (neg)J. Kuroda et al.elderlyMNot available (NA)positive mass location NANANANAnegS. Fatimi et al61FDyspneaRight atrium (RA) +Right ventricle (RV)posExcisional biopsyvariant type- 49, XX, +X, t(8;14)(q24;q32), −14, +der(14)(3;8;14) (q27;q24;q32), +18, +20negPiero Maria Stefani et al.33MDyspneaRAposExcisional biopsyNAnegChang-Fu Peng et al.44FAbdominal DiscomfortRAposPericardial analysist(8;14)negDimitrios et al.77MDyspnealarge mass , location NAposAutopsy of masst(8;14)negTakai K. et al.70MDyspneaRAposExcisional biopsyt(8;14)negM.De Filippo et al.74MDyspneaRAposPericardial analysisNAnegSylvie et al.67Msyncopebi-atrial + LVposExcisional biopsyt(8;14)negFrancesco Santini et al.84MDyspneaNo massposPericardial analysist(8;14)negPresented to our facility DisclosuresNo relevant conflicts of interest to declare.