title: Primary cardiac lymphoma: report of two cases occurring in immunocompetent subjects.
abstract: Primary cardiac lymphomas (PCLs), involving solely heart and/or pericardium at presentation, are rare events. They are frequently recognized at autopsy and generally carry a poor prognosis due either to a delay in the diagnosis or to infiltration of heart structures. We report here on two patients with large B-cell PCL. One is a 52-year-old man who presented with multiple cardiac tumors infiltrating mainly the right atrium and the inter-atrial septum. Diagnosis was established by ultrasound-assisted transesophageal biopsy of the intra-atrial multilobated tumor mass. He was treated with Rituximab-implemented high-dose sequential (R-HDS) chemotherapy followed by autologous peripheral blood stem cell transplantation, attaining complete response. He had no evidence of disease 24 months from onset. The second patient was a 70-year-old woman who presented with pericardial tamponade and low-output cardiac failure. Despite prompt pericadiocentesis and chemotherapy with cyclophosphamide and vincristine, she died 2 weeks later. Postmortem examination revealed large B-cell lymphoma proliferation confined to the heart. Whether primitive heart localizations represent an independent prognostic factor, and what specific measures should be adopted in patients with this rare presentation is the subject of the present report and review of the literature.
authors.affiliation: Hematology and Bone Marrow Transplantation Unit, Ospedale "S. Camillo-Forlanini", Rome, Italy.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Anghel
authors.forename: Valerio
authors.initials: V
authors.lastname: Zoli
authors.forename: Nicola
authors.initials: N
authors.lastname: Petti
authors.forename: Daniele
authors.initials: D
authors.lastname: Remotti
authors.forename: Mariano
authors.initials: M
authors.lastname: Feccia
authors.forename: Paolo
authors.initials: P
authors.lastname: Pino
authors.forename: Ignazio
authors.initials: I
authors.lastname: Majolino
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D004562:Electrocardiography; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007121:Immunocompetence; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation; D000069283:Rituximab; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab