Long-Term Outcomes of Primary Cardiac Lymphoma
Authors: Ibrahim Sultan, Edgar Aranda-Michel, Andreas Habertheuer, Arman Kilic, George Arnaoutakis, Valentino Bianco, Olugbenga Okusanya
Journal: Circulation (2020)

rimary cardiac lymphoma (PCL) is a rare disease with an incidence of 1% of primary cardiac malignancies and 0.5% of all extranodal lymphomas. 1,2hemotherapy continues to be the primary treatment in PCL, but there have been reports of other treatment strategies including radiation, immunotherapy, and surgery. 1,3Unfortunately, there is a paucity of data on PCL.This study aimed to address this lack of data by leveraging a contemporary national database.We used the National Cancer Database to identify all patients with PCL between 2004 and 2016; institutional review board approval was obtained.All patients had confirmed primary tumor site in the heart with a diagnosis of extranodal non-Hodgkin lymphoma, although the coding has not been validated before and is a limitation of this database.Patients were divided into groups on the basis of the year of their diagnosis.Kaplan-Meier survival estimates were generated for the entire cohort.Cox multivariable regression was used to determine factors associated with mortality.We chose 2010 as the middle of the study period because of survival benefit reported in 2010 with the addition of rituximab to the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen. 4 total of 305 patients were identified between 2004 and 2016 with a primary diagnosis of PCL, based on the international classification of diseases for oncology.The majority of patients were White (82%) and male (54.5%).The average age was 68.7±14.2years.The majority of cancers were graded as B cell or pre-B cell (92.7%).The most common Charlson comorbidity index as 0 (59.3%),followed by 1 (23.3%).The most prevalent therapy used (combination or isolated) was chemotherapy (71.8%), followed by immunotherapy (21.6%), surgery (16.7%), and radiation (8.2%).Most patients were treated at an academic (46.9%), comprehensive community (27.9%), or integrated network cancer program (18.4%).Overall survival of the cohort was 46.6% with a median survival of 45.4 (interquartile range, 5.62-120.38)months (Figure ,A).On Cox multivariable regression, increasing age was associated with lower survival (hazard ratio [HR], 1.04 [1.02, 1.05]) while treatment with chemotherapy was associated with improved survival (HR, 0.33 [0.23, 0.46]) (Figure ,B).There was a significant difference in survival estimates when stratified by years of diagnosis (P=0.050), with 43.7%, 40.1%, and 38.1% surviving at 1, 3, and 4 years, respectively, in the 2003 to 2006 cohort, compared with 71.6%, 60.2%, and 53.1%, respectively, in the most recent cohort (Figure ,C).Moreover, the Cochrane-Armitage trend test for the year grouping was significant (P<0.001).If untreated, PCL has a grim prognosis, with survival reported at less than a month.However, with appropriate treatment, survival can be extended to 5 years. 2 The rate of surgery in this study (15%) is lower than the 28% that has been historically reported. 1This trend in management strategy may be attributable to advances in chemo-and immunotherapies, with more than 50% of the contemporary