Primary Cardiac Lymphoma: Italian Multicenter Experience

Primary cardiac lymphoma (PCL), an extranodal lymphoma involving only the heart (with possible involvement of the pericardium) is a rare entity, accounting for 2% of primary cardiac tumors and 0.5% of extranodal lymphomas. It is more frequent in males; clinical presentation is predominated by cardiac symptoms. The most common histology is Diffuse Large B Cell Lymphoma (DLBCL). Being DLBCL the most frequent histology, patients are usually treated with R-CHOP (rituximab - cyclophosphamide, doxorubicin, vincristine and prednisone) or R-CHOP-like chemoimmunotherapy regimens, with an historically poor outcome, although in the last years survival rates significantly increased. Due to the rarity of this condition, isolated case reports and a few reviews have been published so far, that in most cases included a population collected in a wide period of time, heterogeneously managed both in terms of treatments received and follow-up, and who often did not strictly respect the criteria of PCL. Indeed, while some aspects of PCL are well-known, especially the ones concerning its clinical presentation, a few topics deserve more in-depth analysis. The rationale of this study is to provide an overview on PCL in Italy, trying to shed light on unknown aspects of the disease and on unanswered questions about its management that could be helpful in clinical practice.

• Complete remission (CR) [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Overall Response Rate (ORR) [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Progression-Fee Survival (PFS) [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Frequencies of the type of chemo(immuno)therapy and of the number of cycles received as first and second line. [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Frequencies of the type of Central Nervous System (CNS) prophylaxis [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Cumulative incidence rate of Central Nervous System (CNS) relapse detected during treatment or follow-up. [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]
• Characteristics of patients [Time frame: The endpoint will be evaluated within 2 months from the end of data collection. The end of data collection is scheduled within the Q4 (fourth quarter) of 2023.]