Primary Cardiac Lymphoma: Italian Multicenter Experience

The rationale of this study is to provide an overview on PCL (Primary Cardiac Lymphoma) 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.

Study Overview

• Status: Recruiting
• Conditions: Primary Cardiac Lymphoma

Detailed Description

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.

• Study Type: Observational
• Enrollment (Estimated): 43

Contacts and Locations

• Study Contact: Name: Samantha Deianira Dattoli; Phone Number: 0599769914; Email: sdattoli@filinf.it
• Study Contact Backup: Name: Giorgio Priolo; Phone Number: 0131.033175; Email: gpriolo@filinf.it

Study Locations

• Italy
  • Bergamo, Italy, 24127
    • Recruiting
    • Azienda Ospedaliera Papa Giovanni XXIII - Ematologia
    • Principal Investigator: Giuseppe Gritti, MD
    • Contact: Giuseppe Gritti, MD; Email: g.gritti@asst-pg23.it
  • Brescia, Italy, 25123
    • Recruiting
    • ASST Spedali Civili di Brescia - Ematologia
    • Contact: Rosa Daffini, MD; Email: rosadaffini@yahoo.it
    • Principal Investigator: Rosa Daffini, MD
  • Ferrara, Italy, 44124
    • Recruiting
    • Azienda Ospedaliero-Universitaria di Ferrara - Arcispedale Sant'Anna - Ematologia e fisiopatologia della coagulazione
    • Contact: Mariarosaria Sessa, MD; Email: sessamariarosaria@gmail.com
    • Principal Investigator: Mariarosaria Sessa, MD
  • Milan, Italy, 20132
    • Recruiting
    • Istituto Scientifico San Raffaele - Unitа Linfomi - Dipartimento Oncoematologia
    • Principal Investigator: Andrés JM Ferreri, MD
    • Contact: Andrés JM Ferreri, MD; Email: andres.ferreri@hsr.it
  • Milan, Italy, 20133
    • Recruiting
    • Fondazione IRCCS Istituto Nazionale dei Tumori di Milano - Ematologia
    • Contact: Anna Guidetti, MD; Email: anna.guidetti@istitutotumori.mi.it
    • Principal Investigator: Anna Guidetti, MD
  • Milan, Italy, 20162
    • Recruiting
    • ASST Grande Ospedale Metropolitano Niguarda - SC Ematologia
    • Principal Investigator: Erika Ravelli, MD
    • Contact: Erika Ravelli, MD; Email: erika.ravelli@ospedaleniguarda.it
  • Perugia, Italy, 06129
    • Recruiting
    • Ospedale S. Maria della Misericordia - Ematologia
    • Contact: Leonardo Flenghi, MD; Email: leonardo.flenghi@ospedale.perugia.it
    • Principal Investigator: Leonardo Flenghi, MD
  • Pisa, Italy, 56126
    • Not yet recruiting
    • AOU Pisana - U.O. Ematologia
    • Contact: Sara Galimberti, MD; Email: sara.galimberti@med.unipi.it
    • Principal Investigator: Sara Galimberti, MD
  • Roma, Italy, 00168
    • Not yet recruiting
    • Roma - Universitа Cattolica S. Cuore - Ematologia
    • Contact: Stefan Hohaus, MD; Email: stefan.hohaus@Unicatt.it
    • Principal Investigator: Stefan Hohaus, MD
  • Roma, Italy, 00183
    • Recruiting
    • AO Sant'Andrea - Ematologia
    • Principal Investigator: Agostino Tafuri, MD
    • Contact: Agostino Tafuri, MD; Email: agostino.tafuri@ospedalesantandrea.it
  • Terni, Italy, 05100
    • Not yet recruiting
    • A.O. S. Maria di Terni - S.C. Oncoematologia
    • Contact: Anna Marina Liberati, MD; Email: marina.liberati@unipg.it
    • Principal Investigator: Anna Marina Liberati, MD
  • Torino, Italy, 10126
    • Not yet recruiting
    • A.O.U. Citta della Salute e della Scienza di Torino - Ematologia Universitaria
    • Contact: Federica Cavallo, MD; Email: f.cavallo@unito.it
    • Principal Investigator: Federica Cavallo, MD
  • Verona, Italy, 37134
    • Recruiting
    • AOU Integrata di Verona - U.O. Ematologia
    • Contact: Isacco Ferrarini, MD; Email: isacco.ferrarini@univr.it
    • Principal Investigator: Isacco Ferrarini, MD
  • SS
    • Sassari, SS, Italy, 07100
      • Recruiting
      • AOU di Sassari - Ematologia
      • Contact: Claudio Fozza, MD; Email: cfozza@uniss.it
      • Principal Investigator: Claudio Fozza, MD
  • Salerno
    • Pagani, Salerno, Italy, 84016
      • Not yet recruiting
      • Presidio ospedaliero "A. TORTORA" - U.O. Onco-ematologia
      • Contact: Catello Califano, MD; Email: c.califano@aslsalerno.it
      • Principal Investigator: Catello Califano, MD
  • TS
    • Trieste, TS, Italy, 34121
      • Recruiting
      • Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI) - SC Ematologia
      • Contact: Elisa Lucchini, MD; Email: elisa.lucchini@asugi.sanita.fvg.it
      • Principal Investigator: Elisa Lucchini, MD
  • Venezia
    • Mestre, Venezia, Italy, 30174
      • Not yet recruiting
      • Ospedale Dell'angelo - U.O. Ematologia
      • Principal Investigator: Cristina Skert, MD
      • Contact: Cristina Skert, MD; Email: cristina.skert@aulss3.veneto.it

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: N/A

• Sampling Method: Non-Probability Sample

Study Population

Patient affected by primary cardiac lymphoma from 1st January 2000 to 31st December 2020.

Description

Inclusion Criteria:

• Diagnosis of PCL (any histotype) defined as an extranodal lymphoma involving only the heart with possible involvement of the pericardium.
• Age ≥18 years.
• Diagnosis histologically confirmed. Cytofluorimetry analysis of pericardial fluid showing phenotypic features consistent with a primary cardiac lymphoma is permitted if a biopsy sample is not feasible/available for diagnosis; monoclonality is not accepted as a surrogate for diagnosis.
• Date of diagnosis: from 1st January 2000 to 31st December 2020.
• Only patients treated in first-line with chemoimmunotherapy regimens including an anti-CD20 monoclonal antibody are eligible for the study.
• Signed written informed consent (in case of unreachable subject please see chapter 11.2)

Exclusion Criteria:

• Secondary cardiac involvement from lymphoma; primary mediastinal lymphoma with pericardial infiltration or other lymphomas with involvement of the pericardium/the heart by contiguity and primary effusion lymphoma are not included in this study.
• Patients treated with chemotherapy regimens that did not include an anti-CD20 monoclonal antibody as first-line therapy.
• Refuse to sign a written informed consent

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Patients enrolled; Patients with a diagnosis primary cardiac lymphoma. The study includes patients with PCL diagnosed from 01/01/2000 to 31/12/2020.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Overall survival (OS)	Overall survival, the percentage of patients alive of the cohort (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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.

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Complete remission (CR)	The Complete Remission is defined as the lack of detectable evidence of tumor in the cohort of patients (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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)	The Overall Response Rate is defined as the proportion of patients who have a partial or complete response to therapy (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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)	The Progression-Free Survival is the length of time during and after the treatment that patients live with the disease, but it does not get worse (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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.	The numbers of treatment types and numbers of cycles of therapy received by the cohort of patients (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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.	The proportion of patients with disease relapse on Central Nervous System (CNS)	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	Analysis of the following characteristics: Age, gender, disease localization (atria, ventricles, cardiac arteries and veins, pericardium), HIV positivity, type of symptoms at diagnosis.	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	The numbers of treatment prophylaxis types for Central Nervous System administered to the cohort of patients (patients with cardiac lymphoma diagnosed from 01/01/2000 to 31/12/2020).	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.

Collaborators and Investigators

• Sponsor: Fondazione Italiana Linfomi - ETS
• Investigators: Principal Investigator: Elisa Lucchini, MD, Trieste - Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI) - SC Ematologia

Study record dates

Study Major Dates

• Study Start (Actual): October 2, 2024
• Primary Completion (Estimated): April 1, 2026
• Study Completion (Estimated): June 1, 2026

Study Registration Dates

• First Submitted: April 6, 2023
• First Submitted That Met QC Criteria: April 6, 2023
• First Posted (Actual): April 19, 2023

Study Record Updates

• Last Update Posted (Actual): January 5, 2026
• Last Update Submitted That Met QC Criteria: December 31, 2025
• Last Verified: December 1, 2025

More Information

Terms related to this study

• Keywords: PCL
• Additional Relevant MeSH Terms: Neoplasms; Immune System Diseases; Neoplasms by Histologic Type; Lymphatic Diseases; Lymphoproliferative Disorders; Immunoproliferative Disorders; Lymphoma
• Other Study ID Numbers: FIL_Lympho-Heart

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No