Primary Cardiac Burrkit Lymphoma: Characteristics, Clinical Presentation and Outcome
Authors: Jumayli Mohamed , Chen Emily , Sadrzadeh Hossein 
Published: October 2019

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 dyspnea

As 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 1. Age Gender(Male : M, Female : F) Presenting Symptom Mass Location pericardial effusion Diagnosed by Chromosomal abnormalities HIV status Reference 79 F Dyspnea Left Ventricle (LV) Positive (pos) Pericardial analysis variant type-(8,22)(q24,q11), no c-myc negative (neg) J. Kuroda et al. elderly M Not available (NA) positive mass location NA NA NA NA neg S. Fatimi et al 61 F Dyspnea Right atrium (RA) +Right ventricle (RV) pos Excisional biopsy variant type- 49, XX, +X, t(8;14)(q24;q32), −14, +der(14)(3;8;14) (q27;q24;q32), +18, +20 neg Piero Maria Stefani et al. 33 M Dyspnea RA pos Excisional biopsy NA neg Chang-Fu Peng et al. 44 F Abdominal Discomfort RA pos Pericardial analysis t(8;14) neg Dimitrios et al. 77 M Dyspnea large mass , location NA pos Autopsy of mass t(8;14) neg Takai K. et al. 70 M Dyspnea RA pos Excisional biopsy t(8;14) neg M.De Filippo et al. 74 M Dyspnea RA pos Pericardial analysis NA neg Sylvie et al. 67 M syncope bi-atrial + LV pos Excisional biopsy t(8;14) neg Francesco Santini et al. 84 M Dyspnea No mass pos Pericardial analysis t(8;14) neg Presented to our facility

Disclosures

No relevant conflicts of interest to declare.