title: Primary cardiac lymphoma: diagnosis, treatment and outcome in a modern series.
abstract: Primary cardiac lymphoma (PCL) represents a rare subset of extranodal lymphomas for which the primary lesion arises from the heart and/or the pericardium. Fundamental characteristics of PCL remain uncertain, regarding optimal diagnosis strategy, pathological features, treatments, as well as prognostic factors. This is a single-institution retrospective study of patients with histologically proven lymphoma, presenting with exclusive or predominant myocardial invasion at time of diagnosis. Thirteen patients were included, all of whom had symptoms related to cardiac tumour location with chronic chest pain in six (46%), dyspnea in seven (54%) and arythmia in three (23%). Sub-acute and acute congestive heart failure were noticed in respectively nine (70%) and one (9%). PCL was identified at transthoracic echocardiography and computed tomography scan in 80 and 100% of patients, respectively. Most frequent location was the right atrium in 10 (77%) patients. Pericardial effusion was identified in 10 (77%). Pathological diagnosis-diffuse large B-cell lymphoma in 12 cases and Burkitt in 1 case-was made on cardiac surgical biopsies in 9 cases and by intravascular procedure in 2 cases. All patients received first-line chemotherapy, with a complete response rate of 62%. Recurrences occurred in 55% of patients, mostly at extracardiac extranodal sites. Our data confirm that PCL harbours specific clinical and anatomical features. The aggressiveness of PCL mainly results from the possible onset of acute cardiac events. Further molecular characterization may help to further individualize PCL among diffuse and intrathoracic lymphomas. Copyright © 2016 John Wiley & Sons, Ltd.
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service d'Hématologie, Centre Hospitalier Lyon Sud, Pierre Bénite, France.
authors.forename: Sylvain
authors.initials: S
authors.lastname: Carras
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service d'Anatomie Pathologique, Centre Hospitalier Lyon Sud, Pierre Bénite, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Berger
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service d'Anatomie Pathologique, Groupement Hospitalier Est, Lyon, France.
authors.forename: Lara
authors.initials: L
authors.lastname: Chalabreysse
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Laboratoire d'Hématologie, Centre Hospitalier Lyon Sud, Pierre Bénite, France.
authors.forename: Evelyne
authors.initials: E
authors.lastname: Callet-Bauchut
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service de Pneumologie, Hôpital Louis Pradel, Lyon, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service d'Hématologie, Centre Hospitalier Lyon Sud, Pierre Bénite, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
authors.affiliation: Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Service de Pneumologie, Hôpital Louis Pradel, Lyon, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Girard
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: cardiac tumour; chemotherapy; lymphoma; pericarditis; rare tumour